Search

There is limited experience in evaluating abnormal ductus arteriosus (DA) insertion pattern by contrast-enhanced cardiac computed tomography (cardiac CT) in patients with right-sided obstructive cardiac defects. Retrospective review of 38 infants with right-sided obstructive cardiac defects who underwent a preoperative cardiac CT between 2016 and 2021. We reviewed the types of cardiac lesions, patterns of ductal insertion, frequency of pulmonary artery (PA) stenosis requiring intervention, total dose length product (DLP), and effective radiation dose. Of 38 infants, 45% were female, the median gestational age and weight were 37 (range 34–40) weeks and 2.95 (range 2–4) kg. The most common pathologies were pulmonary atresia with ventricular septal defect (24%) and tetralogy of Fallot (24%). The abnormal ductal insertion patterns were DA inserting into the left PA in 39%, DA bifurcating into branch PA in 32%, and DA inserting into the right PA in 13%. Of the 38 infants, 76% developed branch PA stenosis requiring intervention. Among patients with abnormal DA insertion, 44% required branch PA arterioplasty during their index surgery compared to 17% without abnormal DA insertion. Regardless of the type of abnormal DA insertion, 67% developed bilateral branch PA stenosis over time. The mean DLP was 8 mGy-cm and the mean calculated effective radiation dose was 0.312 mSv. The utilization of contrast-enhanced cardiac CT in infants with right-sided obstructive heart defects can offer crucial insights into abnormal ductus arteriosus insertion patterns. This information is valuable for effective procedure planning and for monitoring the development of branch pulmonary artery stenosis. [ABSTRACT FROM AUTHOR]

Introduction The integration of Point of Care Ultrasound (POCUS) into the care of trauma patients, specifically the E-FAST, has improved the accuracy of initial diagnoses and improved time to surgical intervention in critically ill patients. Physician assistants (PAs) are critically important members of any military trauma resuscitation team and are often team leaders in a pre-hospital setting. They may receive training in ultrasound but there are little data to support their use or evaluate their effectiveness in using POCUS. We designed a study to evaluate the image quality of an E-FAST Exam performed by Emergency Medicine Physician Assistant (EMPA) Fellows and Emergency Medicine (EM) Interns following identical training. Our hypothesis is that image quality obtained by EMPAs will be non-inferior to those images obtained by EM Interns. Materials and Methods This is a prospective single-blinded study comparing the image quality of E-FAST exams performed by first year EM interns and first year EMPA fellows. All participants completed standard POCUS training prior to enrollment in the study. A total of 8 EMPAs and 8 EM first year residents completed 10 recorded E-FAST exams to be used as study images. Participants also viewed a 15-question slide show containing images of positive (6) and negative (9) E-FAST exams and recorded their interpretations. Images were reviewed by expert reviewers who were blinded to which images were collected by which group. An image quality score was recorded for each view as well as an overall image quality score. Image quality was rated on a 1 to 5 image quality scale. Results For overall image quality, the mean score for EMPAs was 3.6 ± 0.5 and for EM residents was 3.2 ± 0.5 with statistical significance favoring better image quality from the EMPAs. The time to completion for the EFAST exam for EMPAs was 4.8 ± 1.3 minutes and for interns it was 3.4 ± 1.4 minutes (P value = 0.02). There was no difference in image interpretation quiz scores between the groups (mean score 92% among interns and 95% among PAs). Conclusions POCUS is an imaging modality which is very portable and relatively inexpensive which makes it ideal for military medicine. PAs are essential members of military trauma teams, and often run an initial trauma resuscitation. Being able to correctly identify patients who have free fluid early in the course of treatment allows for more correct evacuation criteria to ensure the sickest patients get to care the fastest. Although there are limited data to support POCUS use by non-physicians, our data support a growing body of evidence that it is not the profession or baseline medical education that determines an individual's ability to use and incorporate ultrasound into bedside and clinical practice. Our study shows that with training and experience PAs or other members of the military health care team can use the EFAST to better care for trauma patients. [ABSTRACT FROM AUTHOR]

Endovascular stenting of the pulmonary artery treats arterial stenosis from pulmonary hypertension, congenital heart defects, or post-transplant stricture. Patients with malignant extrinsic pulmonary arterial compression, secondary to large mediastinal or pulmonary masses, often present with dyspnea, hypoxemia, and right ventricular failure. Conventional therapies like surgery, chemotherapy, and radiation are often slow and fail to promptly resolve acute symptoms.Balloon angioplasty and stenting have been explored as a rapid treatment to alleviate symptoms of external pulmonary artery compression. Despite its potential, the adoption of this procedure is limited due to risks like stent misplacement, migration, cardiac arrhythmias, and arterial rupture.This paper presents 3 cases of pulmonary angiography and stenting performed for malignant extrinsic pulmonary artery compression. These cases aim to demonstrate the feasibility of pulmonary artery stenting, encouraging its consideration as a palliative option for symptomatic patients with this condition.

The article presents a clinical case of development of mural infective endocarditis (IE) with formation of vegetations in the region of the upper third of the interventricular septum in patient A., 51 years old, with uncorrected tetralogy of Fallot. The patient was admitted with complaints of increased heart rate, interruptions in the heart work, feet swelling, shins up to the upper third, dyspnea at rest, general weakness, fever up to 39 оC, sweating, headache. During transthoracic echocardiography on the upper third of the interventricular septum (IVS) from the side of the RV, an additional echo-positive linear mobile formation 0.9 cm long is visualized, similar to the chords of the tricuspid valve. Thus, based on the identification of 1 major Duke criterion (imaging criterion — the presence of parietal vegetation) and 3 minor criteria (predisposing factors — congenital heart disease of the blue type; fever >38 оC; positive blood culture not meeting the requirements for the major criterion), laboratory and instrumental diagnostic data and anamnesis, the following diagnosis was established: "Secondary infective endocarditis with damage to the upper third of the interventricular septum". Taking into account the sensitivity of the identified microflora, polymyxin and moxifloxacin were prescribed. During therapy, a dry cough and dry wheezing in the projection of the middle lobe on the right appeared. To exclude septic embologenic pneumonia, computed tomography of the chest organs was performed, on which no focal/infiltrative changes were detected. However, a shunt connecting the brachiocephalic trunk with the right pulmonary artery was visualized, which may explain the cause of compensation of hypoxemia that arose due to pathological hemodynamics as a result of high pulmonary stenosis. Given the incomplete effectiveness of therapy, as well as the sensitivity of the infectious agents isolated, a decision was made to change antibacterial therapy to vancomycin in combination with imipenem. Conservative treatment methods allowed not only to compensate for the existing clinical symptoms, but also to achieve reference values of laboratory parameters, despite the impossibility of surgical intervention.

Abstract Background Arterial tortuosity syndrome is a rare Autosomal recessive disease that leads to a loss of function of the connective tissues of the body, this happens due to a mutation in the solute carrier family 2 member 10 (SLC2A10) gene. ATS is more likely to occur in Large and medium-sized arteries including the aorta and pulmonary arteries. This syndrome causes the arteries to be elongated and tortuous, This tortuosity disturbs the blood circulation resulting in stenosis and lack of blood flow to organs and this chronic turbulent flow increases the risk of aneurysm development, dissection and ischemic events. Case presentation A 2 years old Arabian female child was diagnosed with ATS affecting the pulmonary arteries as a newborn, underwent a pulmonary arterial surgical reconstruction at the age of 2 years old due to the development of pulmonary artery stenosis with left pulmonary artery having a peak gradient of 73 mmHg with a peak velocity of 4.3 m/s and the right pulmonary artery having a peak gradient of 46 mmHg with a peak velocity of 3.4 m/s causing right ventricular hypertension. After surgical repair the left pulmonary artery has a peak pressure gradient of 20 mmHg, with the right pulmonary artery having a peak pressure gradient of 20 mmHg. Conclusion ATS is a rare genetic condition that affects the great arteries especially the pulmonary arteries causing stenotic and tortuous vessels that may be central branches or distal peripheral branches that leads to severe right ventricular dysfunction and hypertension. We believe that surgical treatment provides the optimum outcomes when compared to transcather approaches especially when the peripheral arteries are involved. Some challenges and hiccups might occur, especially lung reperfusion injury that needs to be diagnosed and treated accordingly.

Pulmonary artery stenosis represents a group of disorders involving main, branch or peripheral pulmonary arteries with pain, dyspnea, hemoptysis or even no symptoms. Early diagnosis and timely intervention are crucial for reducing mortality, but timely diagnosis is challenging due to the non-specific symptoms. Computed tomography pulmonary angiography (CTPA) is useful in the diagnosis because it can provide more details about abnormal changes in the lumen, vessel wall and adjacent mediastinal structures. Congenital and acquired pulmonary artery anomalies have some characteristics on CTPA, which can be useful for differential diagnosis. Awareness of these conditions is important for radiologists. This pictorial review provides an overview of CTPA imaging features of pulmonary artery stenosis. [ABSTRACT FROM AUTHOR]

Background T-wave inversions on electrocardiograms (ECGs) indicate a variety of conditions, such as coronary artery disease, myocarditis, and cardiomyopathy. Pulmonary artery stenosis (PAS) and pulmonary hypertension (PH) may cause right ventricular enlargement and ischaemia, which are reflected as T-wave inversions on ECGs. Continuous ECG monitoring is crucial for detecting dynamic changes indicative of PAS progression and reversal in right heart remodelling. Case summary This report presents the case of a young woman who experienced exertional dyspnoea for 5 years with ECG findings showing T-wave inversions across multiple leads. The patient was diagnosed with PAS and PH caused by Takayasu arteritis (TA). Following three successful balloon pulmonary angioplasty sessions, the patient exhibited significant clinical improvement, including the remission of PAS and PH. Throughout a 59-month cumulative follow-up period, the sustained effectiveness of the treatment was evidenced by the regression of right heart remodelling, as manifested in the normalization of the initially inverted T-waves on the ECG. Discussion Electrocardiogram changes, including right axis deviation, right bundle branch block, a deep S wave in lead I (R/S < 1), and a prominent R wave in lead aVR (R/Q > 1), have been termed PAS syndrome, often linked to TA-associated PAS, especially in young East Asian females. Early diagnosis is crucial but challenging due to atypical symptoms. The non-invasive ECG is vital for detection, with balloon pulmonary angioplasty serving as an effective treatment for TA-induced PAS when surgery is not an option, improving outcomes and potentially reversing right heart remodelling. [ABSTRACT FROM AUTHOR]

Structural variants (SVs) pose a challenge to detect and interpret, but their study provides novel biological insights and molecular diagnosis underlying rare diseases. The aim of this study was to resolve a 9p24 rearrangement segregating in a family through five generations with a congenital heart defect (congenital pulmonary and aortic valvular stenosis and pulmonary artery stenosis), by applying a combined genomic analysis. The analysis involved multiple techniques, including karyotype, chromosomal microarray analysis (CMA), FISH, genome sequencing (GS), RNA-seq, and optical genome mapping (OGM). A complex 9p24 SV was hinted at by CMA results, showing three interspersed duplicated segments. Combined GS and OGM analyses revealed that the 9p24 duplications constitute a complex SV, on which a set of breakpoints matches the boundaries of the CMA duplicated sequences. The proposed structure for this complex rearrangement implies three duplications associated with an inversion of ~ 2 Mb region on chromosome 9 and a SINE element insertion at the more distal breakpoint. Interestingly, this genomic structure of rearrangement forms a chimeric transcript of the KANK1/DMRT1 loci, which was confirmed by both RNA-seq and Sanger sequencing on blood samples from 9p24 rearrangement carriers. Altogether with breakpoint amplification and FISH analysis, this combined approach allowed a deep characterization of this complex rearrangement. Although the genotype–phenotype correlation remains elusive from the molecular mechanism point of view, this study identified a large genomic rearrangement at 9p24 segregating with a familial congenital heart defect, revealing a genetic biomarker that was successfully applied for embryo selection, changing the reproductive perspective of affected individuals. [ABSTRACT FROM AUTHOR]

Williams–Beuren syndrome (WBS) is a congenital multisystem disorder affecting the cardiovascular, central nervous, and musculoskeletal systems. Cardiovascular abnormalities, which consist principally of vascular stenoses, occur in approximately 80% of people with WBS and are the predominant cause of early morbidity and mortality. Supravalvar aortic stenosis and peripheral pulmonary artery stenosis are the most common stenotic lesions in WBS, though other stenoses often occur, including stenoses of the coronary arteries. Approximately one‐third of people with WBS undergo cardiovascular interventions. The risk of sudden cardiac death is markedly higher than the general population, with most events occurring in the periprocedural period. Because of the rarity of WBS and the often‐complex nature of the cardiovascular abnormalities, most physicians, including cardiologists, have limited experience in caring for patients with WBS. Further, heretofore, clinical cardiovascular management guidelines based on international expert consensus have not been available. This state‐of‐the‐art review provides a comprehensive synopsis of the cardiovascular abnormalities in WBS and presents clinical management guidelines based on the authors' expert consensus.

Objectives Neoadjuvant chemotherapy (NACT) is recommended for muscle-invasive bladder cancer, and robust treatment response may result in lack of grossly identifiable tumor in the cystectomy specimen. Current gross examination and reporting protocols, however, do not include specific guidance on the approach to these specimens. Methods A Qualtrics survey was disseminated by email and X (formerly Twitter). Responses from pathologists and pathologists' assistants (PAs) were included. The survey interrogated demographics, practice settings, prevalence of NACT use, approach to gross examination, and reporting practices in the setting of both grossly visible tumor or ulcer bed and the complete absence of a gross lesion. Results Based on 55 respondents' experience, identifying gross tumor occurred less frequently than tumor or ulcer bed (40% vs 71%). Lack of identification of any gross lesions was estimated to occur in 29% of cases. Gross examination practices were relatively consistent in cases with residual gross tumor or gross tumor bed, with agreement that gross tumor should be submitted as 1 block per centimeter (66%), and tumor or ulcer bed should be submitted in its entirety (97%). Gross examination practices appeared more varied when no gross lesions were identified. Overall, most responders stated they "definitely" or "maybe" support a standardized gross examination (89%) and reporting (96%) protocol. Conclusions With the increased use of NACT, lack of any gross lesion leads to inconsistent gross examination techniques. This study provides insight into the current approach to examination of post-NACT cystectomies and suggests that a desire exists among pathologists and pathologists' assistants for more standardized practice. [ABSTRACT FROM AUTHOR]

The widespread introduction of molecular genetic research methods into health care practice has made it possible to diagnose rare microdeletion syndromes in patients with multiple congenital malformations. Aim of the study is to present the results of a literature search and demonstrate a clinical observation of Williams-Beuren syndrome in 10-month-old monochorionic twins with congenital malformations of the cardiovascular system in combination with kidney pathology and an additional spleen. Results. Williams-Beuren syndrome (WBS) is a rare congenital disorder characterized by specific craniofacial dysmorphisms (elphic face) and a hoarse voice in combination with cardiovascular damage, mental retardation, musculoskeletal disorders, and hypercalcemia. WBS occurs in the population with a frequency of 1:7,500-1000 infants. The presence of a specific phenotype is associated with a hemizygous microdeletion of the long arm of chromosome 7 at region 7q11.23. The size of the deletion varies from 1.5 to 1.8 Mb and results in the loss of several neighboring genes. The diagnosis is made syndromologically and confirmed by modern molecular cytogenetic methods. Pathologically significant WBS mutations include loss of the ELN gene and loss of neighboring genes such as LIMK1, RFC2, BAZ1B, GTF2I, STX1A, CLIP2, GTF2IRD, NCF. Haploinsufficiency of ELN gene is the main marker of WBS and causes insufficient synthesis of elastin protein, which leads to development of pathology of heart and blood vessels (elastin arteriopathy), disorders of connective apparatus of joints, abnormalities of vocal cords and skin. LIMK1 hemizygosity is associated with impaired visual-spatial constructive cognition. Deletion of the RFC2 gene can cause growth retardation and developmental delay. Reduced intelligence can be caused by a mutation of the GTF2I gene and hypercalcemia by a mutation of the BAZ1B gene. The phenotypic manifestations of WBS are also thought to be influenced by the reduced expression of flanking intact genes. The diagnosis, treatment, and adjustment of patients with WBS require an interdisciplinary team of specialists. The presented clinical case demonstrates multisystem pathology in 10-month-old monochorionic dizygotic twins in whom Williams-Beuren syndrome was clinically diagnosed and confirmed by FISH: ish del (7)(q11.23q11.23)(ELN-). Conclusion. To confirm the genetic component in congenital multisystem pathology, it is necessary to use modern molecular genetic diagnostic methods. Determination of genetic mutation, its size and origin is important for medical genetic counseling. Early confirmation of the WBS allows to make an individual prognosis of the child's life and development, as well as to determine in time the optimal methods of treatment and adaptation, and to advise the parents in planning the next birth of children in the family. [ABSTRACT FROM AUTHOR]

The widespread introduction of molecular genetic research methods into health care practice has made it possible to diagnose rare microdeletion syndromes in patients with multiple congenital malformations.. Aim of the study is to present the results of a literature search and demonstrate a clinical observation of Williams- Beuren syndrome in 10-month-old monochorionic twins with congenital malformations of the cardiovascular system in combination with kidney pathology and an additional spleen. Results. Williams- Beuren syndrome (WBS) is a rare congenital disorder characterized by specifi c craniofacial dysmorphisms (elphic face) and a hoarse voice in combination with cardiovascular damage, mental retardation, musculoskeletal disorders, and hypercalcemia. WBS occurs in the population with a frequency of 1:7,500-1000 infants. The presence of a specifi c phenotype is associated with a hemizygous microdeletion of the long arm of chromosome 7 at region 7q11.23. The size of the deletion varies from 1.5 to 1.8 Mb and results in the loss of several neighboring genes. The diagnosis is made syndromologically and confi rmed by modern molecular cytogenetic methods. Pathologically signifi cant WBS mutations include loss of the ELN gene and loss of neighboring genes such as LIMK1, RFC2, BAZ1B, GTF2I, STX1A, CLIP2, GTF2IRD, NCF. Haploinsuffi ciency of ELN gene is the main marker of WBS and causes insuffi cient synthesis of elastin protein, which leads to development of pathology of heart and blood vessels (elastin arteriopathy), disorders of connective apparatus of joints, abnormalities of vocal cords and skin. LIMK1 hemizygosity is associated with impaired visual- spatial constructive cognition. Deletion of the RFC2 gene can cause growth retardation and developmental delay. Reduced intelligence can be caused by a mutation of the GTF2I gene and hypercalcemia by a mutation of the BAZ1B gene. The phenotypic manifestations of WBS are also thought to be infl uenced by the reduced expression of fl anking intact genes. The diagnosis, treatment, and adjustment of patients with WBS require an interdisciplinary team of specialists. The presented clinical case demonstrates multisystem pathology in 10-month-old monochorionic dizygotic twins in whom Williams- Beuren syndrome was clinically diagnosed and confi rmed by FISH: ish del (7)(q11.23q11.23)(ELN-). Conclusion. To confi rm the genetic component in congenital multisystem pathology, it is necessary to use modern molecular genetic diagnostic methods. Determination of genetic mutation, its size and origin is important for medical genetic counseling. Early confi rmation of the WBS allows to make an individual prognosis of the child’s life and development, as well as to determine in time the optimal methods of treatment and adaptation, and to advise the parents in planning the next birth of children in the family.

Open in new tab Download slide OBJECTIVES Left pulmonary artery (LPA) or bifurcation stenoses at Fontan palliation can be very challenging to treat and may also require cardioplegia and aortic transection. Moreover, the low pressure of Fontan circulation and the bulkiness of the aorta increase the risk of a patch angioplasty collapse. Pre-Fontan LPA stenting of stenotic LPAs overcomes those drawbacks therefore the present study aimed to evaluate its advantageous impact on Fontan surgery. METHODS A multicentre retrospective analysis was performed on 304 consecutive Fontan patients. The study population was divided into 2 groups (LPA stented, n  = 62 vs not stented, n  = 242); pre-and postoperative data were compared. RESULTS LPA-stented patients had a higher prevalence of systemic right ventricle (P  = 0.01), hypoplastic left heart syndrome (P  = 0.042), complex neonatal palliations (Norwood/Damus–Kaye–Stansel) and surgical LPA patch repair at Glenn (P  < 0.001). No differences were found in cross-clamp rates, early (P  = 0.29) and late survival (94.6% vs 98.4, P  = 0.2) or complications (P  = 0.14). Complex palliations on ascending aorta/aortic arch (P  = 0.013) and surgical LPA repair at Glenn (P  < 0.001) proved to be risk factors for LPA stenting before Fontan at multivariable analysis. CONCLUSIONS The LPA-stented group showed similar outcomes in terms of survival and complications rate compared to patients without LPA stenosis; however, they significantly differ in their higher preoperative risk profile and in their more complex anatomy. Complex neonatal palliations involving ascending aorta or aortic arch may increase the risk of pulmonary branches stenosis requiring stenting; therefore, preoperative stenting of LPA stenoses could help to reduce the surgical risk of complex Fontan procedure by avoiding the need for cross-clamp or complex mediastinal dissections to perform a high-risk surgical repair. [ABSTRACT FROM AUTHOR]

Pulmonary artery stenosis represents a group of disorders involving main, branch or peripheral pulmonary arteries with pain, dyspnea, hemoptysis or even no symptoms. Early diagnosis and timely intervention are crucial for reducing mortality, but timely diagnosis is challenging due to the non-specific symptoms. Computed tomography pulmonary angiography (CTPA) is useful in the diagnosis because it can provide more details about abnormal changes in the lumen, vessel wall and adjacent mediastinal structures. Congenital and acquired pulmonary artery anomalies have some characteristics on CTPA, which can be useful for differential diagnosis. Awareness of these conditions is important for radiologists. This pictorial review provides an overview of CTPA imaging features of pulmonary artery stenosis.

Pulmonary artery coarctation (PACoA) is a major problem that increases the frequency of intervention. However, there is little evidence regarding the prediction of PACoA development. A retrospective chart review was performed on 42 patients who underwent modified Blalock-Taussig shunt and preoperative contrast-enhanced computed tomography. An uneven PA branching was defined as an abnormal ductus arteriosus connection to the left PA distal to the PA branching on contrast-enhanced computed tomography. Nineteen (45.2%) of 42 patients were diagnosed with PACoA. The median diameters of the ductus on the aorta and PA sides were 4.1 mm and 3.6 mm in the PACoA group and 3.6 mm and 2.9 mm in the non-PACoA group, respectively (P =.07 and.28, respectively). Tortuous ductus was recognized in 7 (36.8%) patients in the PACoA group and 14 (60.8%) patients in the non-PACoA group (P =.12). PACoA was associated with pulmonary atresia (16 patients [84.2%] in the PACoA group and 12 patients [52.1%] in the non-PACoA group) (P =.02). All 19 patients had uneven PA branching in the PACoA group, whereas 5 of 23 (21.7%) patients had uneven PA branching in the non-PACoA group (P <.001). Uneven PA branching rather than the ductus arteriosus size was strongly associated with PACoA development; therefore, morphologic assessment by contrast-enhanced computed tomography should be considered in patients with pulmonary atresia. [Display omitted] [ABSTRACT FROM AUTHOR]

Stents are tubular meshed endoprostheses implanted mini-invasively through a transcatheter intervention to guarantee the patency of body conduits, mainly in cardiovascular applications. In pediatric cardiology, stenting has become an accepted procedure in the treatment of congenital heart disease (CHD) as an alternative to open-heart surgery. CHD refers to a range of defects affecting the heart's structure and function arising from abnormal development during pregnancy. While during fetal life, the presence of additional shunts allows for the establishment of parallel circulation and survival of gestation, CHD is not compatible with extrauterine life, and medical intervention is required soon after birth. This review aims to discuss the state of the art of stenting in CHD. Despite the severity of these pathologies, investment from the industry remains limited due to the restricted number of cases, and dedicated devices are still missing. As a consequence, commercially available adult stents are commonly exploited on an off-label basis in newborns without any optimization for the specific anatomy and required function. In this review, a classification of the available stents is provided, resuming the manufacturing technologies, materials, and geometrical aspects to obtain the target biomechanical performance. After analyzing the fetal circulation, different forms of CHD amenable to stenting are considered, collecting the stents currently adopted and discussing the clinical outcomes to outline the features of an ideal device. [ABSTRACT FROM AUTHOR]

Open in new tab Download slide OBJECTIVES The aim of this study was to more accurately predict the optimal pulmonary artery (PA) reconstruction procedure (Lecompte manoeuvre or original Jatene procedure) during the arterial switch operation, we focused on the horizontal sectioning (HS) angle between the left hilum PA and the great arteries using preoperative computed tomography imaging. METHODS We defined the HS angle α (β) as the angle between the tangential line from the posterior (anterior) wall of the left PA at the hilum to the left anterior (right posterior) surface of the main PA and the tangential line from the left surface of the ascending aorta to the left anterior (right posterior) surface of the main PA. We identified 14 consecutive patients diagnosed with transposition of the great arteries or transposition of the great artery-type double-outlet right ventricle who underwent preoperative computed tomography imaging. The original Jatene or Lecompte procedure was used for 9 (OJ group) and 5 (L group) patients. Relationships of the great arteries of the OJ and L groups were side by side in 8 and 2 patients, oblique in 1 and 1 patient and anteroposterior in 0 and 2 patients, respectively. RESULTS In the OJ group, β was greater than α in all patients. The median α/β value was 0.618. In group L, α was greater than β in all patients. The median α/β was 1.307. Left PA stenosis caused by stretching was not observed in the L group. Coronary obstruction was not identified in the OJ group. Left PA stenosis behind the neo-ascending aorta was observed in 1 patient in the OJ group and required reoperation. CONCLUSIONS The HS angle may be a useful predictor of optimal intraoperative PA reconstruction during arterial switch operation, especially for side-by-side or oblique relationships. [ABSTRACT FROM AUTHOR]

The bone impaction grafting through femoral head–neck fenestration was a favorable hip preservation procedure but without prognosis estimation. This study retrospectively reviewed 79 patients' clinical data (114 hips) with osteonecrosis of the femoral head (ONFH) who underwent this procedure from June 2009 to June 2019. By the end of June 2022, the median survival time of the hip was (74.13 ± 44.88) months, and the success rate of hip preservation was 68.42%. Lateral reserved angle (LPA) and combined reserved angle (CPA) had statistically significant differences (P  < 0.001) both in univariate analysis and a multivariate logistic regression model. The multivariate logistic regression model of area under curve (AUC) area of the receiver operating characteristic (ROC) curve was 0.931(sensitivity = 95.00%, specificity = 88.40%, log-rank test: P  < 0.01), and the calibration curve indicated good prediction accuracy. The ROC analysis and Cox proportional hazards regression model revealed that the cutoff point of LPA was 50.95° (sensitivity = 95.00%, specificity = 72.09%, log-rank test: P  < 0.05) and the cutoff point of CPA was 90.51° (sensitivity = 90.00%, specificity = 90.70%, log-rank test: P  < 0.05). A nomogram plot to predict the risk of failure (C-index = 0.873, 95% CI: 0.785 to 0.961) and nomograms for predicting the survival probability at 1, 2 or 3 years whose calibration curves showed excellent prediction accuracy were available for the clinician. Preserved angles (PAs) are valuable in the prediction of prognosis in surgical treatment. The bone impaction grafting through femoral head–neck fenestration can achieve better clinical efficacy, especially for patients with LPA >50.95° and CPA >90.51°. [ABSTRACT FROM AUTHOR]

Not all stents are suitable for children. For instance, premounted stents can be used in infants and small children but cannot dilate with age to accommodate adult-sized pulmonary arteries. Conversely, the Pul-Stent adapts to somatic growth. Thus, our hospital implemented the Pul-Stent in pediatric patients with branch pulmonary artery stenosis. This study summarizes our initial experience with Pul-Stents in this patient population, including the efficacy and safety. We implanted 37 Pul-Stents in 35 patients between August 2014 and June 2015. The patients' mean age and weight at stent implantation were 6.7 ± 3.0 years and 20.9 ± 8.7 kg, respectively. Bench testing revealed that axial shortening of the Pul-Stent was minimal with further dilation, and the radial strength did not change. The stents were successfully deployed in all cases, except two with minor malpositioning. Primarily, 8–12 mm mounting balloons were used for the initial implantation, and a long sheath (8–10 F) was used for delivery. After stent implantation, the minimal lumen diameter in the stenosed segment increased by 50% in 97% (34/35) of patients. Furthermore, the pressure gradient across the stenosed segment decreased by 50% in 77% (23/30) of biventricular patients. One stent fracture and one stent restenosis were noted during the follow-up visits (mean follow-up time: 4.6 ± 1.7 years). Eighteen patients (51%) underwent repeat catheterization; ten had successful redilation. No aneurysms or stent fractures were observed. Our initial results indicate that the Pul-Stent is safe and effective in pediatric patients and can be further dilated over time to accommodate somatic growth. Moreover, the Pul-Stent has good compliance and adequate radial strength to treat pulmonary artery stenosis effectively. [ABSTRACT FROM AUTHOR]

Abstract Aims The prognosis is poor for patients with pulmonary artery stenosis‐associated pulmonary hypertension (PAS‐PH). Identifying predictors of prognosis in PAS‐PH is crucial to preventing premature death, which has rarely been investigated. We aimed to explore the cardiopulmonary exercise testing (CPET) parameters to predict the prognosis of these patients. Methods We prospectively included all patients with PAS‐PH who underwent CPET between September 2014 and June 2021 in Fuwai Hospital (ClinicalTrials.gov ID: NCT02061787). The primary outcome was clinical worsening, including death, rehospitalization for heart failure, or deterioration of PH. Results Seventy‐two patients were included in this study. A median of 2‐year follow‐up revealed that 18 (25%) patients experienced clinical worsening. The 1‐year, 3‐year, and 5‐year event‐free survival rates were 92.5%, 81.7%, and 62.7%, respectively. Patients with clinical worsening demonstrated significantly worse baseline haemodynamics and poorer exercise capacity than their counterparts. Multivariable Cox regression identified that peak O2 pulse could independently predict clinical worsening [hazard ratio: 0.344, 95% confidence interval (CI) 0.188–0.631, P

Coronary bifurcation lesions and treatment with two-stent techniques have been developed, including the double kissing (DK) crush technique. The use of this technique in children or noncoronary vessels, including pulmonary arteries, has not been described. We present a 12-year-old girl with Alagille syndrome, a ventricular septal defect (VSD), and complex bilateral pulmonary artery (PA) stenoses who is status post six catheterizations for PA angioplasty and stenting to improve her marked right ventricular hypertension. With collaboration between the congenital and structural teams, she successfully underwent the DK crush technique for a complex lesion in her PA. This improved pulmonary flow and allowed for successful surgical VSD closure.

Since the first lung transplantation in humans was performed in 1963, patient selection, standardized procurement, and surgical techniques have been developed and established for this procedure. However, despite these developments, surgical complications continue to be important factors influencing patient morbidity and mortality, and efforts should be made to decrease morbidity and improve survival rates by understanding, rapidly detecting, and appropriately treating surgical complications.

Alagille syndrome, caused by mutations in the gene encoding Jagged1 (JAG1), a ligand in the Notch signaling pathway, is an autosomal dominant disorder with developmental abnormalities affecting the liver, heart, eyes, face and skeleton. The aim of the present study is try to disclose the clinical features, management and outcomes of pulmonary artery stenosis associated with Alagille syndrome. By comprehensive literature retrieval, 38 articles involving 401 patients were recruited for this study. The pertinent variables closely related to pulmonary artery stenosis in patients with Alagille syndrome were comprehensively analyzed by following the PRISMA guidelines. The management of pulmonary artery pathologies, especially a severe type of pulmonary artery stenosis in Alagille syndrome, is a concerned matter. Publications of literature retrieval of recent 3 decades were the study material of this article. The pulmonary artery pathologies, especially the severe type of pulmonary artery stenosis in Alagille syndrome, warrant surgical or interventional treatments. After the procedures, the right ventricular to left ventricular pressure ratio was reduced by 25%. There were no intergroup differences in terms of recovery, reintervention and mortality rates between interventionally and surgically treated patients. Transcatheter treatment is preferable due to less trauma. Surgical treatment of pulmonary artery stenosis can be performed currently with intracardiac defect repair.

A 58-year-old patient was admitted to the emergency department due to severe respiratory insufficiency. Anamnesis revealed that the patient had experienced increasing stress dyspnea for a few months. Upon imaging, an acute pulmonary embolism was excluded, but peribronchial and hilar soft tissue proliferation with compression of central parts of the pulmonary circulation was found. The patient had a history of silicosis. The histology report showed tumor-free lymph node particles with prominent anthracotic pigment and dust depositions without evidence of IgG4-associated disease. The patient was administered steroid therapy and underwent simultaneous stenting of the left interlobular pulmonary artery and the upper right pulmonary vein. As a result, a significant improvement in symptoms and physical performance was achieved. The diagnosis of inflammatory or, in particular, fibrosing mediastinal processes can be challenging and important clinical symptoms must be taken into account, especially if the pulmonary vasculature is involved. In such cases, the possibility of interventional procedures should be examined in addition to drug therapy options.

Williams syndrome, and various elastin protein mediated arteriopathies, presents a clinical challenge to pediatric cardiovascular specialists. In the severest phenotypes, multilevel obstruction to the systemic and pulmonic arterial systems result in biventricular dysfunction which can be imminently life-threatening. As a longstanding, quaternary referral center for complex pulmonary arteriopathies and pediatric connective tissue disease, Stanford Medicine Children's Health has developed a sizeable experience managing these patients. This manuscript is a summary of our current strategies, with a focus on our surgical techniques, peri-procedural considerations on timing and staging of various interventions, and long-term results. [ABSTRACT FROM AUTHOR]

Aims: The prognosis is poor for patients with pulmonary artery stenosis‐associated pulmonary hypertension (PAS‐PH). Identifying predictors of prognosis in PAS‐PH is crucial to preventing premature death, which has rarely been investigated. We aimed to explore the cardiopulmonary exercise testing (CPET) parameters to predict the prognosis of these patients. Methods: We prospectively included all patients with PAS‐PH who underwent CPET between September 2014 and June 2021 in Fuwai Hospital (ClinicalTrials.gov ID: NCT02061787). The primary outcome was clinical worsening, including death, rehospitalization for heart failure, or deterioration of PH. Results: Seventy‐two patients were included in this study. A median of 2‐year follow‐up revealed that 18 (25%) patients experienced clinical worsening. The 1‐year, 3‐year, and 5‐year event‐free survival rates were 92.5%, 81.7%, and 62.7%, respectively. Patients with clinical worsening demonstrated significantly worse baseline haemodynamics and poorer exercise capacity than their counterparts. Multivariable Cox regression identified that peak O2 pulse could independently predict clinical worsening [hazard ratio: 0.344, 95% confidence interval (CI) 0.188–0.631, P < 0.001], outperforming other parameters. Peak O2 pulse correlated with PH severity. Incorporating peak O2 pulse into the simplified 2015 European Society of Cardiology/European Respiratory Society risk stratification improved the accuracy for predicting clinical worsening (pre vs. post area under the curve: 0.727 vs. 0.846, P < 0.001; net reclassification index: 0.852, 95% CI 0.372–1.332, P < 0.001; integrated discrimination index 0.133, 95% CI 0.031–0.235, P = 0.011). Conclusions: The prognosis is poor for PAS‐PH, and exercise intolerance and ventilation inefficiency are commonly observed. Peak O2 pulse independently predicted the prognosis of these patients. A low peak O2 pulse identified patients at high risk of clinical deterioration and served for risk stratification of PAS‐PH. [ABSTRACT FROM AUTHOR]

ObjectiveBalloon angioplasty (BA) has been the treatment of choice for pulmonary artery stenosis (PAS) in children. There remains, however, a significant proportion of resistant lesions. The ultra-high pressure (UHP) balloons might be effective in a subset of these lesions. In this study, we analyzed the safety and efficacy with short- to mid-term follow-up results of UHP BA for PAS in children with congenital heart defects (CHD) in our center.MethodsThis is a retrospective cohort study in a single tertiary heart center. Children diagnosed with PAS associated with CHD were referred for UHP BA. All data with these children were collected for analysis with updated follow-up.ResultsA total of 37 UHP BAs were performed consecutively in 28 children. The success rate was 78.4%. A significantly (P = 0.005) larger ratio of the balloon to the minimal luminal diameter at the stenotic waist (balloon/waist ratio) was present in the success group (median 3.00, 1.64–8.33) compared to that in the failure group (median 1.94, 1.41 ± 4.00). Stepwise logistic regression analysis further identified that the balloon/waist ratio and the presence of therapeutic tears were two independent predictors of procedural success. The receiver operating characteristic curve revealed a cut-off value of 2.57 for the balloon/waist ratio to best differentiate success from failure cases. Signs of therapeutic tears were present in eight cases, all of whom were in the success group. Perioperative acute adverse events were recorded in 16 patients, including 11 pulmonary artery injuries, three pulmonary hemorrhages, and two pulmonary artery aneurysms. During a median follow-up period of 10.4 (0.1–21.0) months, nine cases experienced restenosis at a median time of 40 (4–325) days after angioplasty.ConclusionsThe UHP BA is safe and effective for the primary treatment of PAS in infants and children with CHD. The success rate is high with a low incidence of severe complications. The predictors of success are a larger balloon/waist ratio and the presence of therapeutic tears. The occurrence of restenosis during follow-up, however, remains a problem. A larger number of cases and longer periods of follow-up are needed for further study.

Coronary bifurcation lesions and treatment with two-stent techniques have been developed, including the double kissing (DK) crush technique. The use of this technique in children or noncoronary vessels, including pulmonary arteries, has not been described. We present a 12-year-old girl with Alagille syndrome, a ventricular septal defect (VSD), and complex bilateral pulmonary artery (PA) stenoses who is status post six catheterizations for PA angioplasty and stenting to improve her marked right ventricular hypertension. With collaboration between the congenital and structural teams, she successfully underwent the DK crush technique for a complex lesion in her PA. This improved pulmonary flow and allowed for successful surgical VSD closure. [ABSTRACT FROM AUTHOR]

Williams-Beuren syndrome (WBS) is a congenital multisystem disorder affecting the cardiovascular, central nervous, and musculoskeletal systems. Cardiovascular abnormalities, which consist principally of vascular stenoses, occur in approximately 80% of people with WBS and are the predominant cause of early morbidity and mortality. Supravalvar aortic stenosis and peripheral pulmonary artery stenosis are the most common stenotic lesions in WBS, though other stenoses often occur, including stenoses of the coronary arteries. Approximately one-third of people with WBS undergo cardiovascular interventions. The risk of sudden cardiac death is markedly higher than the general population, with most events occurring in the periprocedural period. Because of the rarity of WBS and the often-complex nature of the cardiovascular abnormalities, most physicians, including cardiologists, have limited experience in caring for patients with WBS. Further, heretofore, clinical cardiovascular management guidelines based on international expert consensus have not been available. This state-of-the-art review provides a comprehensive synopsis of the cardiovascular abnormalities in WBS and presents clinical management guidelines based on the authors' expert consensus.

Objectives: Pulmonary artery (PA) bifurcation stenosis often requires simultaneous stent placement, which may be technically challenging. Limited data exist regarding this practice in infants. We aim to report the procedural outcomes and safety of bifurcation stent placement in infants., Methods: We performed a single-center retrospective review of infants younger than 12 months who underwent simultaneous stent placement for PA bifurcation stenosis from January 1, 2001 through December 31, 2019., Results: Seventeen infants underwent simultaneous PA bifurcation stent placement. The median age was 6.4 months (1.1-10.1 months), and weight was 5.8 kg (3-10.6 kg). Nine (52.9%) patients had had prior PA intervention. Most stents were placed in central PAs (28, 82.4%), followed by lobar branches (6, 17.6%). All patients received pre-mounted stents. The peak gradient across each branch decreased from 47.4 ± 16 to 18.7 ± 13 mm Hg (P less than .0001). The right ventricle to systemic systolic pressure ratio decreased from systemic (1.0 ± 0.3) to just over half systemic (0.58 ± 0.2) (P = .0001). The minimum vessel diameter increased from 3.6 ± 1.5 to 6.0 ± 1.9 mm (P less than .0001). There were 4 (23.5%) patients with high severity adverse events. There were no procedure-related deaths. The median follow-up period was 83.8 months (5.3 months-19.4 years). All patients had subsequent PA re-intervention at a median time of 8.1 months (2.9 months-8.8 years), and median time to re-operation was 19.1 months (2.9 months-7.5 years)., Conclusions: Simultaneous PA stent placement is an effective strategy for relief of bifurcation stenosis in infants. Future transcatheter interventions are necessary to account for patient growth, but may delay the need for re-operation.

Stents are tubular meshed endoprostheses implanted mini-invasively through a transcatheter intervention to guarantee the patency of body conduits, mainly in cardiovascular applications. In pediatric cardiology, stenting has become an accepted procedure in the treatment of congenital heart disease (CHD) as an alternative to open-heart surgery. CHD refers to a range of defects affecting the heart’s structure and function arising from abnormal development during pregnancy. While during fetal life, the presence of additional shunts allows for the establishment of parallel circulation and survival of gestation, CHD is not compatible with extrauterine life, and medical intervention is required soon after birth. This review aims to discuss the state of the art of stenting in CHD. Despite the severity of these pathologies, investment from the industry remains limited due to the restricted number of cases, and dedicated devices are still missing. As a consequence, commercially available adult stents are commonly exploited on an off-label basis in newborns without any optimization for the specific anatomy and required function. In this review, a classification of the available stents is provided, resuming the manufacturing technologies, materials, and geometrical aspects to obtain the target biomechanical performance. After analyzing the fetal circulation, different forms of CHD amenable to stenting are considered, collecting the stents currently adopted and discussing the clinical outcomes to outline the features of an ideal device.

BackgroundPulmonary artery stenosis is often associated with congenital heart disease. The aim of the study was to evaluate the efficacy and safety of stenting for branch pulmonary artery stenosis using a biodegradable nitride iron stent (IBS® Angel™) and right ventricular systolic and diastolic function.MethodsFrom July 2021 to February 2022, a total of 11 cases (ages ranged from 36 to 86 months old) were included in this pre and post-intervention, prospective, cohort and preclinical study. All cases underwent transthoracic echocardiographic (TTE), chest radiography, along with computed tomography (256-slice scanner, multiple-detector) and right heart catheterization. Different types of biodegradable nitride iron stents were implanted. TTE was performed serially 1 day, 1 month and 3 months after the procedure to evaluate the rate of restenosis and right ventricular function.ResultsStenting was successful in 11 patients. There were no major adverse cardiovascular events related to the device or to the procedure. Blood perfusion in the branch pulmonary artery was improved immediately. At follow-up, there was no significant restenosis that required re-intervention. None of the patients suffered from in-stent thrombosis, vascular embolism, stent displacement or heart failure. Compared with normal values, there were statistical with regards to FAC, E/A and E′/A′. Furthermore, we found that TAPSE correlated significantly with pulsed Doppler S wave (p = 0.008) and left ventricular ejection fraction (p < 0.01). The early trans-tricuspid inflow velocities E/E′ (tissue doppler at the lateral tricuspid annulus) correlated significantly with E′/A′ (p = 0.009). FAC and E′/A′ were statistically different from those prior to stenting (p = 0.041 and p = 0.035) when tested one month postoperatively. At three months postoperatively, only E/A showed a statistical difference (p = 0.015).ConclusionOur analysis suggests that biodegradable nitride iron stents are feasible, safe, and effective in children. Some small improvements were observed in right ventricular systolic and diastolic function after successful transcatheter intervention, although change was not statistically significant due to the small sample number. (A clinical Trial to Evaluate the Safety and Efficacy of IBS Angel in Patients With Pulmonary Artery Stenosis (IRIS); NCT04973540).

Objective: To investigate the long-term efficacy of percutaneous transluminal pulmonary angioplasty (PTPA) in patients with Takayasu arteritis (TA) and pulmonary artery stenosis and pulmonary hypertension (PH). Methods: Data from 183 lesions from 79 surgeries performed on 32 patients with TA and PH were analyzed. Symptoms, laboratory investigation results, World Health Organization (WHO) functional class, 6-min walk distance (6 MWD), hemodynamic parameters, and prognosis were analyzed at baseline and follow-up. Results: The mean (± SD) age of the 32 patients (28 female, 4 male) was 42.8 ± 11.9 years, and the median follow-up was 49.5 months (interquartile range, 26–71 months). Compared with baseline, changes in total bilirubin, N-terminal pro-brain natriuretic peptide (NT-proBNP) level, 6 MWD, and WHO score functional class demonstrated significant differences (P <0.001). Echocardiography findings, right and left ventricular diameter, tricuspid annular plane systolic excursion, and estimated pulmonary artery systolic pressure were all improved (P =0.016, P <0.001, P <0.001, and P =0.005, respectively). Importantly, repeat right heart catheterization revealed that mean pulmonary artery pressure, pulmonary vascular resistance, and cardiac index also improved significantly at follow-up (P <0.001, P <0.001, and P =0.011, respectively). Pulmonary angiography revealed post-procedure restenosis in 64 (35.0%) lesions underwent PTPA within three to six months. Among three patients who underwent stent implantation, one experienced restenosis. Two patients died during the follow-up period, one from aggravation of right heart failure after lung infection, and the other in a traffic accident. Conclusions: Results of this study indicated that PTPA significantly improved clinical symptoms, exercise tolerance, and hemodynamic parameters in patients with TA pulmonary artery stenosis and PH. More importantly, reperfusion pulmonary edema significantly decreased, and no patient died of PTPA-related complications with guidance from the pressure wire. [ABSTRACT FROM AUTHOR]

Objective: We present prenatal diagnosis of familial 2p15 microduplication associated with pulmonary artery stenosis, single umbilical artery and left foot postaxial polydactyly on fetal ultrasound. Case report: A 34-year-old woman underwent amniocentesis at 17 weeks of gestation because of advanced maternal age. Amniocentesis revealed the karyotype of 46,XX. Prenatal ultrasound examination at 21 weeks of gestation showed pulmonary artery stenosis, single umbilical artery and left foot postaxial polydactyly. Repeat amniocentesis was performed at 22 weeks of gestation and array comparative genomic hybridization (aCGH) analysis on the DNAs extracted from amniocytes revealed the result of arr 2p15 (61, 495, 220–62,885,679) × 3.0 [GRCh37 (hg19)] with a 1.391-Mb 2p15 duplication encompassing seven Online Mendelian Inheritance in Man (OMIM) genes of USP34, XPO1, FAM161A, CCT4, COMMD1, B3GNT2 and TMEM17. aCGH analysis on the DNAs extracted from parental bloods confirmed a familial transmission from a normal carrier mother who had no phenotypic abnormality. A 3270-g female baby was delivered at term with mild pulmonary artery stenosis and left foot postaxial polydactyly. The infant had normal physical and psychomotor development when follow-up at age of one year. Conclusion: Prenatal diagnosis of fetal structural abnormalities should include aCGH analysis in addition to conventional cytogenetic analysis.

Background: Pulmonary artery stenosis (PAS) is common in congenital heart disease (CHD). The indication for treatment of PAS is primarily based on invasively measured pressure gradients. Anesthetics used during cardiac catheterization cause systemic and pulmonary hypotension. We hypothesize that this leads to underestimation of right ventricular (RV) pressure load and under treatment of PAS. The aim of this study is to describe the use of epinephrine to unmask RV pressure load in patients with PAS. Methods: All cardiac catheterizations in which epinephrine was administered to evaluate RV load were included. There was an indication for treatment in case of a right to left ventricular (RV:LV) pressure ratio >0.6. The indication for treatment before and after epinephrine was evaluated to determine its role in clinical decision making. Results: A total of 74 procedures were included. In all procedures the invasively measured LV pressure was below the awake blood pressure. At baseline, 33 patients had a RV:LV ratio >0.6. In 41 patients the baseline RV:LV ratio was 0.6 thereby revealing the indication for treatment. No epinephrine related complications were registered. Conclusion: The hypotensive properties of anesthetics during cardiac catheterization may lead to underestimation of RV pressure load. Invasive pressure measurements should be performed under conditions similar to awake conditions. Epinephrine provocation prevented under treatment in 25% of our patients.

ObjectiveTo investigate the long-term efficacy of percutaneous transluminal pulmonary angioplasty (PTPA) in patients with Takayasu arteritis (TA) and pulmonary artery stenosis and pulmonary hypertension (PH).MethodsData from 183 lesions from 79 surgeries performed on 32 patients with TA and PH were analyzed. Symptoms, laboratory investigation results, World Health Organization (WHO) functional class, 6-min walk distance (6 MWD), hemodynamic parameters, and prognosis were analyzed at baseline and follow-up.ResultsThe mean (± SD) age of the 32 patients (28 female, 4 male) was 42.8 ± 11.9 years, and the median follow-up was 49.5 months (interquartile range, 26–71 months). Compared with baseline, changes in total bilirubin, N-terminal pro-brain natriuretic peptide (NT-proBNP) level, 6 MWD, and WHO score functional class demonstrated significant differences (P

Williams syndrome (WS) is an arteriopathic derangement associated with supravalvular aortic stenosis and branch pulmonary stenosis. We describe double-outlet right ventricle with mitral atresia and aortic arch hypoplasia in an infant with WS. This case demonstrates the difficulty in managing patients with WS with complex cardiac defects. To our knowledge, this is the first reported single-ventricle physiology in a patient with WS. (Level of Difficulty: Advanced.)

Endovascular stenting of the pulmonary artery treats arterial stenosis from pulmonary hypertension, congenital heart defects, or post-transplant stricture. Patients with malignant extrinsic pulmonary arterial compression, secondary to large mediastinal or pulmonary masses, often present with dyspnea, hypoxemia, and right ventricular failure. Conventional therapies like surgery, chemotherapy, and radiation are often slow and fail to promptly resolve acute symptoms. Balloon angioplasty and stenting have been explored as a rapid treatment to alleviate symptoms of external pulmonary artery compression. Despite its potential, the adoption of this procedure is limited due to risks like stent misplacement, migration, cardiac arrhythmias, and arterial rupture. This paper presents 3 cases of pulmonary angiography and stenting performed for malignant extrinsic pulmonary artery compression. These cases aim to demonstrate the feasibility of pulmonary artery stenting, encouraging its consideration as a palliative option for symptomatic patients with this condition., (© 2024 The Authors. Published by Elsevier Inc. on behalf of University of Washington.)

Background: Stent implantation has become standard of care in older children and adults for treatment of branch pulmonary artery stenosis (BPAS) and coarctation aorta (CoAo). There are no stents approved or available for infants that have the potential to be dilated to adult diameters. The Minima stent was designed to fulfill this unmet need., Methods: Multicenter, prospective, nonrandomized early feasibility study evaluating safety and effectiveness of the Minima stent for treatment of BPAS and CoAo. Primary endpoints included: (1) successful deployment across lesion, (2) stenosis relief defined by an increase in angiographic diameter of >50% and (3) freedom from stent explant, embolization or migration at 30 days and 6 months., Results: Between 2/2022 and 5/2022, 10 pts underwent Minima stent implantation with a median age and weight of 9 months (4-43 months) and 7.6 kg (5.1-16.9 kg). Procedural success and predefined stenosis relief was achieved in all cases (CoAo [n = 4], BPAS [n = 6]). Adverse events occurred in 3 pts: transient diminished lower extremity pulse (n = 2), distal stent on-balloon displacement successfully managed in the catheterization suite (n = 1). There were no deaths or major adverse events. All patients were free from stent explant and migration at 30 days and 6 months with no evidence for significant restenosis at latest follow-up., Conclusions: Implantation of the Renata Minima stent was safe and effective for the treatment of BPAS and CoAo in this small cohort of infants and young children during early follow-up. Based on these early results, an expanded study with longer follow-up is warranted., (© 2024 The Authors. Catheterization and Cardiovascular Interventions published by Wiley Periodicals LLC.)

In this article, we describe a case of a 33-year-old female with Alagille syndrome complicated by bilateral branch pulmonary artery stenosis resulting in moderate pulmonary hypertension, end-stage liver disease complicated by portal hypertension, and chronic renal disease who presented for combined liver-kidney transplant. Alagille syndrome is an autosomal dominant disease affecting the liver, heart, and kidneys. Multidisciplinary preoperative evaluation was performed with a team consisting of a congenital heart disease cardiologist, a cardiac anesthesiologist, a nephrologist, and a transplant surgeon. We describe Alagille syndrome and our intraoperative management. To our knowledge, this is the first description of a combined liver-kidney transplant in an adult patient with Alagille syndrome. [ABSTRACT FROM AUTHOR]

Now a days due to improved paediatric cardiac care we see patients with repaired or unrepaired congenital heart diseases in adult age more often. Pulmonary artery (PA) stenosis is a common association with Tetrology of Fallot (TOF). It is common to see some degree of residual pulmonary artery stenosis post intra cardiac repair. These stenosis can, sometimes, progress and present in adulthood. Management of patients with peripheral or branch PA stenosis have primarily been catheterization with surgical management of these cases reserved for limited indications. Here we report a case of Right PA ostial stenosis in an adult patient with history of surgical correction of TOF in childhood. We also describe the procedural aspects in the correction of this defect. We here discuss the indications, contraindications and complications of transcatheter and surgical approach. Lack of practice guidelines and recommendations with respect to patient selection, hardware selection, follow up, antiplatelet regimen and operative hemodynamics makes this procedure difficult to learn, monitor and follow. [ABSTRACT FROM AUTHOR]