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Pulmonary balloon valvuloplasty (PBV) is the treatment of choice for subjects with isolated pulmonary valve stenosis (IPS). The purpose of this study was to define fetal echocardiographic features associated with an inpatient PBV prior to newborn hospital discharge and characterize resource utilization of IPS fetuses among participating centers. Six center, retrospective case series of singleton fetuses identified between 2010 and 2020 with IPS. Third-trimester echocardiogram data was compared with postnatal data, included pulmonary valve Doppler velocities, pulmonary valve insufficiency and ductus arteriosus flow direction. Comparison between subjects who underwent inpatient PBV during their newborn hospital admission versus those infants referred for outpatient PBV after initial hospital discharge. We analyzed data by logistic regression, student t test and Chi-Square testing with a p value of ≤ 0.05 considered statistically significant. Forty-nine IPS fetuses were identified. Thirty-eight (78%) underwent inpatient PBV at 5 (range 1–58) days and 11 (22%) underwent outpatient PBV at 51.8 (11–174) days. Newborns requiring an inpatient PBV were more likely to have one or more characteristics on 3rd-trimester fetal echocardiogram: left to right or bidirectional ductus arteriosus flow (61% vs 0%), and/or a peak pulmonary valve velocity > 3.0 m/s (odds ratio 16.9, 95% confidence interval 3.02–94.17) with a sensitivity of 90.4% and specificity of 97.7%. Ductus arteriosus flow direction and pulmonary valve peak velocity in the 3rd trimester can successfully predict the need for newborn inpatient PBV. We speculate these findings may be useful in choosing delivery site for the pregnancy complicated by fetal IPS. [ABSTRACT FROM AUTHOR]

Dysfunctional right ventricular outflow tracts after a repair for tetralogy of Fallot using a transannular patch offer limited nonsurgical opportunities due to their large dimensions. A discrete subannular narrowing between a dilated right ventricle and the enlarged pulmonary trunk was a potential anatomical target for the creation of a landing zone using a prestent in a young male with severe pulmonary regurgitation and moderate stenosis. Asymmetric expansion of the prestent in the angulated outflow tract led to distal stent embolization that was stabilized by another telescoping stent before successful valve implantation. This manuscript details the unique challenges posed by a dilated regurgitant outflow tract for implanting a pulmonary valve. [ABSTRACT FROM AUTHOR]

A case study of 9-month-old 6.2-kg sexually intact male Cavalier King Charles Spaniel with pulmonary valve stenosis was referred for cardiac consultation regarding treatment with balloon valvuloplasty is presented. Topics include Transthoracic echocardiography was repeated at facility by a cardiology resident (AVC), and physical examination of the dog revealed a regularly irregular heart rhythm.

We present the case of an 18‐year‐old man with aborted sudden cardiac death. His initial echocardiogram suggested an anomalous origin of the left coronary artery from the pulmonary artery. Diagnosis was confirmed with coronary angiography. He underwent Takeuchi procedure and fully recovered. A two‐year follow‐up echocardiogram showed a moderate supravalvular pulmonary stenosis related to the transpulmonary baffle. The presence of extensive collateral circulation should raise suspicion of ALCAPA. Postoperative surveillance in this group of patients needs to be oriented in finding complications such as supravalvular pulmonary stenosis, aortic and pulmonary valve insufficiency, and baffle obstruction and leaks. [ABSTRACT FROM AUTHOR]

Percutaneous balloon pulmonary valvuloplasty is the preferred therapy for pulmonary valve stenosis. However, the designs of the cylindrical balloons historically used for valvuloplasty have limitations, especially in patients who have large pulmonary annular diameters. The hourglass-shaped V8 Aortic Valvuloplasty Balloon may prove to be an effective alternative. The balloon has 2 large bulbous segments that are separated by a narrowed waist. The geometric shape is maintained throughout inflation, improving fixation and enabling broader leaflet opening. We present our first experience with the V8 balloon in 3 adults who had severe, symptomatic pulmonary valve stenosis. In addition to describing their cases, we detail our sizing technique for pulmonary valvuloplasty with the V8 balloon. Our successful results suggest that the V8 balloon is efficient and safe for balloon pulmonary valvuloplasty in adults with severe pulmonary valve stenosis. [ABSTRACT FROM AUTHOR]

Background: Congenital pulmonary valve stenosis and patent ductus arteriosus are common congenital heart defects in dogs. However, concurrence of atypical pulmonary valve stenosis and patent ductus arteriosus is uncommon. This report describes the anatomic, histopathologic, and echocardiographic features in a dog with concomitant pulmonary valve stenosis and patent ductus arteriosus with atypical pulmonary valve dysplasia that included a fibrous band of tissue. Case presentation: A 1.5-year-old intact female Chihuahua dog weighing 3.3 kg presented with a continuous grade VI cardiac murmur, poor exercise tolerance, and an intermittent cough. Echocardiography indicated pulmonary valve stenosis, a thickened dysplastic valve without annular hypoplasia, and a type IIA patent ductus arteriosus. The pulmonary valve was thick line-shaped in systole and dome-shaped towards the right ventricular outflow tract in diastole. The dog suffered a fatal cardiac arrest during an attempted balloon pulmonary valvuloplasty. Necropsy revealed pulmonary valve dysplasia, commissural fusion, and incomplete opening and closing of the pulmonary valve because of a fibrous band of tissue causing adhesion between the right ventricular outflow tract and the dysplastic intermediate cusp of the valve. Conclusions: A fibrous band of tissue between the right ventricular outflow track and the pulmonary valve should be considered as a cause of pulmonary valve stenosis. Pulmonary valve stenosis and patent ductus arteriosus can have conflicting effects on diastolic and systolic dysfunction, respectively. Therefore, beta-blockers should always be used carefully, particularly in patients with a heart defect where there is concern about left ventricular systolic function. [ABSTRACT FROM AUTHOR]

To evaluate the long-term cumulative incidence of ≥ moderate pulmonary regurgitation (PR) and re-intervention following balloon pulmonary valvuloplasty (BPV). While BPV for pulmonary valve stenosis (PS) relieves obstruction acutely, long-term outcomes are not well documented. Between 1982 and 2002, 211 patients had BPV for isolated PS. Follow-up data were available for 103 patients. Cumulative incidence of ≥ moderate PR and re-intervention was evaluated and risk factors for ≥ moderate PR assessed in univariate and multivariable analyses. Median age at BPV was 0.7 years (range 1 day-42.2 years); peak catheter gradient was 65 mmHg (range 31-169 mmHg); 23% had critical PS. Sixty-two patients had a recent echocardiogram with median follow-up 15.1 years (range 10.1-26.3 years); 60% had ≥ moderate PR. Three patients had pulmonary valve replacement following BPV due to symptomatic severe PR. In univariate analysis, critical PS, younger age, smaller BSA, and smaller pulmonary annulus at the time of BPV, as well as greater baseline PS gradient by catheterization, were associated with ≥ moderate PR (all p < 0.05). In multivariable analysis, only BSA < 0.3 m was independently associated with ≥ moderate PR (adjusted odds ratio 6.4, 95% confidence interval 1.2-33.6). In the largest study to date of > 10-year outcomes following BPV, 60% of patients with available follow-up data developed ≥ moderate PR. Few patients had pulmonary valve replacement. Patients with lower BSA at the time of BPV were more likely to have greater PR at late follow-up. [ABSTRACT FROM AUTHOR]

In the case presented, a young horse was referred for further evaluation based on a presumptive diagnosis of pulmonic valve endocarditis, bronchopneumonia and a cranial mediastinal abscess based on thoracic ultrasound, echocardiography and clinical signs. However, further echocardiographic evaluation revealed that the abscess was compressing the right ventricular outflow track causing outflow obstruction, a murmur associated with right ventricular outflow tract obstruction (acquired pulmonic stenosis) and right ventricular hypertrophy. Vegetative endocarditis was not present. Medical management was unsuccessful, but surgical drainage and long-term antimicrobial therapy were effective and the horse returned to a successful racing career. [ABSTRACT FROM AUTHOR]

Background A tissue valve is the prosthesis of choice in the majority of children and adults requiring pulmonary valve replacement (PVR). Mechanical valves, on the other hand, are more durable but require anticoagulation therapy and carry the elevated risk of thrombosis. The aim of this study was to investigate the long-term outcomes of mechanical prosthetic valves in a single referral tertiary center. Patients and Methods Recorded data of 121 patients who underwent mechanical PVR between April 2003 and April 2013 at our center were reviewed, retrospectively. Eighty four patients (69.4%) were male and their mean age was 23.12 ± 7.86 years. Tetralogy of Fallot was the most common diagnosis (n = 109). Complete follow-up was performed for all patients (mean 7.02 ± 1.90 years). Results Nobody died and no significant bleeding event was detected during follow-up. Mechanical valve malfunction happened in 10 patients (8.3%) while in 1 of them malfunction happened because of pannus formation and in the remaining 9 because of thrombosis. Just one of the thrombotic events could not be treated with streptokinase and required reoperation. Freedom from reoperation rates were 100, 99, and 98% at 1, 5, and 10 years, respectively; freedom from valve thrombosis event rates were 100, 93, and 91% at 1, 5, and 10 years, respectively; and freedom from bleeding event rates was 98% at 1, 5, and 10 years. Conclusion Mechanical prosthetic valves demonstrated excellent durability and a lowrisk of valve thrombosis at the pulmonary position when patients are adequately anticoagulated. [ABSTRACT FROM AUTHOR]

Pulmonary valve (PV) infective endocarditis (IE) is an extremely rare disease that involves normal as well as abnormal valves. This condition mostly occurs in patients with underlying predisposing factors. It couldbemissedif patients donotpresent with typical features of right-sided endocarditis or in the absence of classic risk factors. The case presented here did not have any known risk factors for IE until surgery and presented mainly with fever, weight loss and musculoskeletal symptoms. [ABSTRACT FROM AUTHOR]

Background: After initial right ventricular outflow tract reconstruction, replacement of the pulmonary valve (PVR) with a bioprosthetic valve may be performed. Bioprosthetic valves fail (PVF) and require repeat replacement. Identification of risk factors for PVF would be useful for clinicians choosing among various options for the initial PVR. Methods: We retrospectively analyzed outcomes of 169 consecutive patients (55% male) with repaired tetralogy of Fallot or pulmonary stenosis undergoing a first PVR. Data were abstracted from the medical records, including gender, diagnosis, indication for PVR, age at PVR (< 10 years or ≥ 10 years), type of valve, and time of PVF. Actuarial freedom from PVF was compared by log rank and parametric survival analysis. Risk factors for PVF were analyzed by univariate and multivariate methods. Prosthesis types for PVR were pulmonary homograft in 56, stented porcine valve in 16, stented porcine valve in Dacron (DuPont, Wilmington, DE) conduit in 26, and bovine pericardial valve in 71. Results: Indication for PVR was pulmonary stenosis in 21% and insufficiency in 79%. Median follow-up for the entire cohort was 8 years. PVF occurred in 24 patients at a median time of 5.7 years. Actuarial freedom from PVF at 10 years was 72% for all valve types, 55% for porcine valve in Dacron conduit, 60% for homograft, 75% for porcine valve, and 78% for bovine pericardial valve (p = 0.36). By univariate analysis, young age (p < 0.0001), male gender (p = 0.0017), and indication of pulmonary stenosis (p = 0.015) were risk factors for PVF. In multivariate analysis, tetralogy of Fallot anatomy (p < 0.06), younger age (p < 0.02), and use of a homograft valve (p < 0.02) were risk factors for early PVF (<3 years). Young age (p < 0.0001) at time of PVR was associated with late PVF. Conclusions: Freedom from reoperation for PVR during 10 years of follow-up is excellent. Younger age, tetralogy of Fallot, and use of a homograft valve were risk factors for early PVF. Only younger age at PVR was a significant risk factor for late PVF. [Copyright &y& Elsevier]

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The article presents a histological study of right ventricle- pulmonary artery valved conduit implantation (RPVC) in dogs with pulmonic stenosis (PS). The feasibility of the RPVC using a homograft in PS model dogs was evaluated histologically. Eight dogs underwent pulmonary artery banding (PAB). After 12 weeks, they were assigned to PAB (n=4) group which does not receive any treatment during the experimental period, and PAB+RPVC (n=4) group which underwent RPVC. After 1 year of PAB, hearts and conduits were explanted from euthanized dogs and were evaluated. The ratios of myocardial fibrosis on both right ventricle (RV) and left ventricle (LV) epicardials, median and endocardial layers were significantly lower in the PAB+RPVC group compare to the PAB group.

From 1991 to 2003 were studied 33 cases with absent pulmonary valve syndrome (AVPS): 66% were female, with a medium age of 1.5 years old and 11 kg of weight. Twenty seven cases (82%) were associated to Tetralogy of Fallot. Fourteen patients (5 younger than 1 year old) had corrective surgery. After the surgery, one patient required ballon pulmonary valvuloplasty for pulmonary stenosis; another one required surgery for changing the pulmonary prothesis one and five years after the first surgery. The rest of the patients did not present important problems. The five year survival was 95.4% in patients older than 6 months and 30.1% in younger patients (p = 0.000). As factors associated to mortality were the age younger than six months old (p = 0.003) and mechanical ventilation (p = 0.001) in our population. We suggest to delay the surgery in this group of patients because no survival were seen with or without the surgery. In older children with symptoms, the surgery also must be delayed in order to avoid more interventions for changing the pulmonary prothesis. [ABSTRACT FROM AUTHOR]

Atrioventricular septal defects constitute 4% of all congenital cardiac malformations. Patients with complete atrioventricular septal defect rarely survive for decades without surgical treatment. Pulmonary stenosis can provide a delicate balance between the pulmonary and systemic circulations and thereby increase longevity. We present the case of a 49-year-old woman whose complete atrioventricular septal defect and associated pulmonary stenosis were diagnosed only after she had given birth to 10 live children through uneventful spontaneous delivery. We discuss her successful surgical treatment in terms of the available medical literature. [ABSTRACT FROM AUTHOR]

Pulmonary valve stenosis (PVS) is one common post-operative problem in patients with tetralogy of Fallot (TOF) after repair. Congenital bicuspid pulmonary valve (BPV) is a condition of valvular stenosis, and the occurrence of congenital BPV is often associated with TOF. Compared with the biomechanical simulation model of the bicuspid aortic valve, the BPV is often neglected. In this study, we developed a dynamic biomechanical model of a simulated normal pulmonary root (PR) with tri-leaflet and a model of simulated PR with BPV in patients with repaired TOF in order to describe the effect of geometric structure with BPV on the stress and strain distribution. The geometry of PR included valvular leaflets, valsalva sinuses, inter leaflet triangle and annulus. Mechanical properties of PV leaflet were obtained from biaxial testing of human PV leaflet, and characterized by an anisotropic hyperelastic material model. Our model simulated complete cardiac cycles to observe valve leaflet dynamic stress/strain behaviors. Our results indicated that stress/strain distribution patterns of normal PV and the BPV were similar on pulmonary root and valve leaflets, but their values were different. When the valve was completely closed, maximum stresses were found leaflet attachment boundary, with their values at 17.1 kPa and 17.2 kPa respectively. When the valve was fully open, maximum stresses were found at the vicinity of commissures of sinus and leaflet, that is, near the annulus, with the values at 115.0 kPa and 143.0 kPa respectively. Compared with normal PR, the valve orifice area in the completely opened position in congenital BPV is significantly reduced. Our initial results demonstrated that geometrical variations with BPV may be a potential risk factor linked to occurrence of PVS in patients with repaired TOF. Computational models could be a useful tool in identifying possible linkage between valve disease development and biomechanical factors. Large-scale clinical studies are needed to validate these preliminary findings. [ABSTRACT FROM AUTHOR]

In 1967, Watson described children in three families with pulmonary valve stenosis, dull intelligence, short stature, and cafe-au-lait spots. We present the case of an undiagnosed adult with coronary artery disease, the Watson syndrome characteristics, and a pulmonary artery aneurysm secondary to pulmonic stenosis. [Copyright &y& Elsevier]

The article presents a case study of a 42-year-old woman with Graves' disease suffered a multi-level airway stenosis due to persistent myhre syndrome and was given treatments such as propranolol, propylthiouracil and hydrocortisone.