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In patients with univentricular heart, the Fontan procedure is the final palliation. This is usually staged. A systemic-to-pulmonary artery shunt is performed in the presence of episodes of cyanotic spells in the neonatal period or in some patients in infancy; a bidirectional superior cardiopulmonary anastomosis is preferred early in life. This is followed by the final Fontan palliation on an elective basis later. For an effective bidirectional superior cavopulmonary anastomosis and Fontan palliation, good-sized confluent pulmonary arteries (PAs) are mandatory in addition to favorable hemodynamic data. Patients with discontinuous PAs that are small in size present a surgical challenge at initial palliation as the one described in this report. [ABSTRACT FROM AUTHOR]

Background: Pulmonary regurgitation is imminent after transannular patch (TAP). We analyze the long-term performance of untreated autologous pericardium (UAP) as valve substitute at pulmonary position in patients requiring TAP.Material and Methods: This cross-sectional study include patients operated between 2007 and 2012 (n = 92). A sample of 19 patients was selected for this study which had a follow-up of more than 3 years. This includes patients with no TAP (n = 4) and with TAP and valve substitute, a monocusp (n = 11) or a tricuspid valve (n = 4) at neopulmonary annulus. Patients underwent echocardiography for assessment of right ventricle function and 18 fluoro-deoxyglucose PET CT scan for measurements of valve substitute at neopulmonary annulus. The target to blood ratio (TBR) of uptake of glucose by monocusp was measured at the cooptation edge of the neopulmonary valve.Results: The median age of the patients is 14 (9 - 37). RV function is preserved (TAPSE 18.9 (10.6 - 22.8)) at a mean follow-up of 4 years (3-9). The measurements of monocusp shows a shrinkage in height of the cusp by 35.5% (70% - 1.0%) and length by 7% (-44% - +104%). There was less shrinkage observed in patients below 15 years of age. The TBR of monocusp was 0.945 (0.17 - 3.35) with a strong correlation between the TBR values of aortic valve leaflet and monocusp leaflet of same patient.Conclusion: The UAP is functional and successful as a valve substitute at neo pulmonary annulus at long-term follow-up. It has resisted calcification and has shown uptake of glucose in physiological limits. [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]

Introduction : Transcatheter pulmonary valve implantation has been an effective treatment for dysfuntional right ventricular tract outflow tract (RVOT). Defining a landing zone before the intervention is crucial in patients with native RVOT. Improper sizing and undefined landing zone will lead to embolization. Methods: It is a retrospective observational study from August 2020 to December 2020 in native RVOT. Three patients who had significant Right ventricle dilatation were analyzed. The multi-slice computed tomography (MSCT) with magnetic resonance imaging and angiography data of all patients before the procedure were analyzed. All patients underwent an angiogram in the same sitting, before the procedure to assess the landing zone, valve diameter as well as the risk for coronary compression. We chose a valve based on valve area 23%-25% more than the area at the waist during balloon sizing. Results: All three patients underwent successful valve implantation. Valve sizes used were 27.5 mm in one and 32 mm in the other two. The mean RVOT gradient postprocedure was 11.5 mm Hg and pre procedure was 43 mmHg. There were no complications during the procedure or at a mean follow-up of 3.6 months. Conclusion: The balloon sizing gives the true narrowest diameter in comparison with MSCT, and increasing this area by 23%-25% will give the appropriate valve size for successful implantation. [ABSTRACT FROM AUTHOR]

We present a case of successful balloon pulmonary valvuloplasty (BPV) in a 37-year-old female with severe valvular pulmonary stenosis with peak gradient of 82 mmHg with history of exertional presyncope. The crux of our case was that patient also had associated mild supravalvular pulmonary stenosis which did not preclude a successful BPV outcome. The patient had rare Bombay negative blood group, and the requisite units were not available across all the blood banks in the state to subject the patient for surgical correction. Although the art of BPV is dying among budding young interventionists, this simple procedure is as good as surgical pulmonary valvotomy so far as the immediate and late outcome is concerned and the presence of mild supravalvular pulmonary stenosis does not preclude a successful outcome. Our case is a unique illustration of feasibility of successful balloon pulmonary valvuloplasty in the presence of associated mild supravalvular stenosis. [ABSTRACT FROM AUTHOR]

Background and Objectives : Infective endocarditis (IE) involving the native pulmonary valve (PV) is extremely rare, with no data in Indian literature. The objective of this communication is to describe the clinical and diagnostic characteristics, underlying risk factors, microbiological features, and management of PVIE. Methods : This is a retrospective analysis of 8 cases of PVIE managed in a tertiary care center from 1992 to 2020. Results : PVIE was observed in 8 patients with underlying congenital cardiac malformation (Group A, 6 Patients) and in patients with central venous catheter (Group B, 2 patients). All the patients had prolonged febrile illness accompanied by right heart failure 4 (50%), septic pulmonary emboli 2 (25%), and pulmonary regurgitation 3 (37.5%). Trans-thoracic echocardiography demonstrated the vegetations, whereas computed tomography of chest diagnosed pulmonary emboli in 2 (25%), and pulmonary artery aneurysm in 1 (12.5%) patient. The early mortality was extremely high (5, 62.5%). Delayed diagnosis, fulminant septicemia, and multi-organ failure resulted in unfavorable outcomes. Conclusions : IE of the native PV is a rare and potentially lethal illness. Diagnosis should be considered in any febrile patient with an underlying congenital defect, central venous line, bacteremia, and comorbidities. Multi-modality imaging should be utilized to enhance the diagnostic yield and detect complications promptly. [ABSTRACT FROM AUTHOR]

Objectives: Adult patients undergoing tetralogy of Fallot (TOF) repair have a higher risk of mortality compared to pediatric patients. Pulmonary regurgitation (PR) further predisposes these patients to heart failure, arrhythmias, and sudden death. Pulmonary valve replacement (PVR) may improve the symptoms in these patients but, fails to reverse the other deleterious effects. Aim of our study was to evaluate the effect of concomitant PVR with TOF repair on right ventricular (RV) parameters, cardiopulmonary exercise capacity, and bioprosthetic valve durability at mid-term. Materials and Methods: Between January 2013 and August 2018, 37 adolescents and adults with TOF who had hypoplastic pulmonary annulus underwent concomitant TOF repair with PVR at our institute. We retrospectively collected the data from the hospital records including follow-up. Results: Mean age of the patients was 18.48 ± 7.53 years. Bioprosthetic valve size ranged from 19 mm to 25 mm. There was no early or late mortality. No patient had developed significant perioperative complications. At a mean follow-up of 53.3 ± 16.4 months, there was no significant change in mean QRS duration, RV function, RV end-systolic and end-diastolic dimensions, RV myocardial performance index, and functional status (including NYHA class and 6-min walk test) compared to at-discharge values. Four patients developed prosthetic valve degeneration with mild PR and without significant increase in gradient. Conclusion: Concomitant PVR with TOF repair in adult provides excellent mid-term outcome, with a minimal rate of pulmonary valve degeneration. It not only eases the early postoperative course but also preserves the RV function as well as functional status at mid-term. [ABSTRACT FROM AUTHOR]

Background: Transcatheter valves provide a safe and effective alternative to surgery for treating dysfunctional right ventricular outflow tracts (RVOTs). We present our early multicenter experience of percutaneous pulmonary valve implantation (PPVI) using Melody valve (Medtronic Inc., Minneapolis, MN). Methods: Patients with stenosed conduits or degenerated bioprosthetic valves in RVOT with combined stenosis and regurgitation were evaluated for suitability of Melody valve implantation. After undergoing an initial structured training, PPVI using Melody transcatheter pulmonary valve (TPV) was guided by an approved proctor. Conduits were serially dilated and prestented with careful coronary interrogation, and bioprosthetic valves were dilated with high-pressure balloons. Clinical and echocardiographic follow-up was performed at 6 monthly intervals. Results: Fifteen patients (three females) aged 23.1 ± 9.5 years in NYHA Class II-III underwent Melody TPV implantation in four Indian centers. The underlying anatomy comprised surgically implanted bioprosthetic valves for pulmonary regurgitation (n= 5), conduit repair for pulmonary atresia (n = 4), Rastelli repair (n = 3), truncus (n = 1), and Ross procedure ( n = 2). Twelve patients had more than one previous surgery. Doppler gradient decreased from 74.2 ± 21.5 mmHg to 10.2 ± 4.5 mmHg after the PPVI. At a median follow-up of 14 months (1-39 months), all the patients were in NYHA Class I with echocardiographic gradients of 8 ± 5.7 mmHg with no evidence of pulmonary regurgitation. There were no major procedural adverse events or deaths. Conclusions: Our early experience shows encouraging results of the PPVI program in India with proctored case selection and meticulous planning. It also confirms the safety and efficacy of Melody TPV for treating dysfunctional RVOT in postoperative patients. [ABSTRACT FROM AUTHOR]

Background : During repair of tetralogy of fallot (TOF) we modified surgical strategies to preserve the valve and annulus if the pulmonary valve leaflets are pliable and not significantly dysplastic. Methods : Initially, the repair was done from the main pulmonary artery (Group-1, 215 patients) and later through an additional incision in the infundibulum of the right ventricle (Group-2, 73 patients). Recently, we changed the approach to commissurotomy of the fused leaflets by releasing the supra valvar tethering and delamination of the cuspal apparatus till the base to improve the mobility of the cusps and do a controlled commissurotomy (Group-3, 14 patients). With delamination, we could extend the limit of the repair to a z-score of -3.5. Results : There was no hospital mortality; two patients died at home after discharge. A mean follow-up of 42.01 months ± 19.25 is available for 198 patients (92%) for group 1, 16.03 ± 7.45 for group 2, and 4.07 ± 2.09 for group 3. The re-intervention-free survival is 94.4% in group 1. The z value improved from -3 (-3-2) to -1.2 (-3 - 0), P = 0.001 in Group 1, from -2.8 (-3-2.4) to -1 (-1.1-0.7), P = 0.001 in Group 2 and from --3 (-4-3) to -1, P = 0.001 in Group 3. In all the groups, there was trivial or mild pulmonary regurgitation. Conclusions : During repair of TOF, adequate valve/annulus sparing is possible if the repair is done from both the main pulmonary artery and infundibular incisions using the delamination technique. [ABSTRACT FROM AUTHOR]

Background: Transcatheter pulmonary valve implantation (TPVI) is a surgical alternative for correcting dysfunctional right ventricular outflow tract conduits in previously operated patients. MyVal transcatheter heart valve (THV) (Meril Life Sciences, India), a new transcatheter valve designed for aortic position has not been used for TPVI. Methods: Patients with stenosed dysfunctional conduits from the right ventricle to pulmonary artery (RV-PA) were prestented after initial computed tomography and balloon interrogation before the implantation of MyVal. Size of MyVal was chosen based on the final diameter of the prestent. Procedural details and post-TPVI follow-up were analyzed. Results: Seven patients aged 17-60 years (median 26 years) had stenosed RV-PA conduits implanted 5-17 years (median 9 years) ago for tetralogy of Fallot in three, following Ross procedure in two, repair of pulmonary stenosis, and following PA debanding in one patient each. Prestenting improved the conduit diameter from 9.3 ± 2.8 mm to 20.8 ± 1.1 mm and relieved the gradient from 87.3 ± 31.7 mmHg (50-137 mmHg) to 12.7 ± 6.4 mmHg (5-20 mmHg). A 23 mm MyVal was implanted in all the seven patients successfully; one patient needed an additional 24.5 mm MyVal valve in valve implantation for residual regurgitation. The mean fluoroscopic time and dose area product were 38.7 ± 25.3 min and 66.917 ± 39.211Gray. cm2, respectively. At a median follow-up duration of 16 months (10-22 months), all patients were asymptomatic receiving dual antiplatelet therapy with no PR and the gradient was 12.5 ± 5.8 mmHg on echocardiography. Although one patient needed an additional valve-in-valve implantation, there were no valve-related adverse events. Conclusions : Early experience of TPVI with MyVal THV in prestented conduits is encouraging with procedural success in all patients and acceptable mid-term outcomes. [ABSTRACT FROM AUTHOR]

Background : Intra-cardiac repair for tetralogy of Fallot has some degree of residual right ventricular outflow tract (RVOT) obstruction. However, the measurement of this gradient intra-operatively might get affected by the depth of anesthesia which is important for the long-term outcome. Aims : The primary aim was to compare intraoperative RVOT gradient post repair under two different anesthetic depths of 1% and 2% end-tidal sevoflurane. The secondary objective was to follow up the changes in RVOT gradient till 1 month postoperatively. Design : Observational study. Setting : Advanced Cardiac Centre of PGIMER, Chandigarh. Methods : Following intracardiac repair, RVOT gradient was measured directly by placing needle into the right ventricle and pulmonary artery at sevoflurane 1%, and subsequently, at 2% end.tidal concentration while maintaining hemodynamic stability. These gradients were also measured using transesophageal echocardiography (TEE) (ClinicalTrials.gov NCT03234582). Results : Twenty-one patients were included in this study that had intra-cardiac repair, of which pulmonary annulus was preserved for 15 cases. Mean RVOT gradients measured invasively and by TEE at end-tidal sevoflurane concentration of 1% and 2% were not significantly different (6.67 ± 4.16 mmHg vs. 6.76 ± 3.82 mmHg, P > 0.05 invasively and 13.01 ± 7.40 mmHg vs. 12.53 ± 7.11 mmHg, P > 0.05 by TEE, respectively). RVOT gradient measured by trans-thoracic echocardiography (TTE) postoperatively at the time of extubation and during follow-up at 1 month showed significant reduction (11.37 ± 6.00 mmHg, P < 0.05 and 9.23 ± 4.92 mmHg, P < 0.01 respectively). Six patients who underwent repair with transannular patch had significant pulmonary regurgitation (PR) following surgery, with no significant change in PR severity or RVOT gradient on increasing anesthetic depth. Conclusions : Postoperative RVOT gradient was not altered by changing depth of anesthesia provided systemic blood pressure was maintained. One month postrepair RVOT gradients were significantly reduced as compared to the intraoperative values. [ABSTRACT FROM AUTHOR]

Background: Absent pulmonary valve syndrome (APVS) is a variant of tetralogy of Fallot characterized by aneurysmal pulmonary arteries, which compresses the tracheobronchial tree, leading to respiratory symptoms. We report the mid term outcomes of surgical correction of patients with APVS. Subject and Methods: A total of 27 patients underwent surgery between 2001 and 2015, and they were followed up for a mean period of 6.4 ± 4.1 years. Out of the 27 patients, 14 (51.9%) were infants. The median age at repair was 9.8 months. Preoperative intubation was required in six patients (22.2%), and 11 patients (40.7%) had symptoms of respiratory distress. The pulmonary valve was replaced with a valved conduit in 15 patients (55.6%), monocusp valve in 6 patients (22.2%), and a transannular patch in 6 patients (22.2%). Reduction pulmonary arterioplasty was done in all patients. Results: The overall 10 year survival was 82.1%. There was 81.1% overall freedom from re intervention at 10 years. No statistically significant difference was found in 10-year survival (P = 0.464) and reoperation rates (P = 0.129) between valved conduit, monocusp, or transannular patch techniques. Older children had statistically significantly longer survival (P = 0.039) and freedom from re intervention (P = 0.016) compared to infants. Patients without respiratory complications had 100% 10 year survival and 93.3% freedom from reoperation at 10 years compared to 55.6% and 60.1%, respectively, for patients with respiratory complications. Conclusion: There has been improvement in surgical results for APVS over the years. However, it still remains a challenge to manage infants and patients with persistent respiratory problems. [ABSTRACT FROM AUTHOR]

Isolated absent pulmonary valve syndrome with an intact ventricular septum (APVS/IVS) is an extremely rare lesion. The prenatal diagnosis was described in a few reports. In the current work, we report a unique fetal case with this rare anomaly. The family counseling for isolated APVS/IVS is a different challenging process than with fetal Fallot type diagnosis. Moreover, 22q11 deletion has never been found as an association. Parents must also be aware of the expected early heart failure in cases with large patent ductus arteriosus and the need for its closure as early as possible. [ABSTRACT FROM AUTHOR]

Background and Objectives: The main effect of pulmonary stenosis is a rise in right ventricular pressure. This pressure overload leads to multiple changes in the shape, dimensions, and volumes of the right ventricle (RV) that are reversed after the relieve of the valve obstruction. We thought to study the changes in the RV in patients undergoing balloon pulmonary valvuloplasty (BPV) using three-dimensional (3D) echocardiography. Subjects and Methods: The study included 50 patients with isolated valvular pulmonary stenosis who underwent BPV at our hospital from December 2016 to August 2017; echocardiography was recorded preprocedural and 3 months after the procedural. Results: The median age of the study group at the time of the procedure was 2.7 years. The indexed RV wall thickness, basal, and mid-right ventricular dimensions decreased significantly after the procedure (P < 0.005), and the longitudinal dimension increased significantly after the procedure (P < 0.005). The end-systolic and the end-diastolic volumes (EDVs) by 3D echocardiography increased insignificantly (P > 0.05), and the right ventricular function increased significantly (P < 0.05), indicating that the changes in the EDVs were more than the changes in the end-systolic volumes. Conclusion: There are several factors that interplay together and result in reverse remodeling of the RV after BPV including regression in the RV hypertrophy; changes in the interventricular septal morphology, bowing, and mobility; and changes in the ventricular geometry and dimensions, rather than changes in the ventricular volumes. [ABSTRACT FROM AUTHOR]

Hemolytic anemia and right ventricular outflow tract obstruction following device closure of ruptured sinus of Valsalva have seldom been reported in isolated case reports, and exact incidence is not known. A gentleman presented with severe delayed hemolytic anemia following the use of cocoon duct occluder for ruptured sinus of Valsalva. Right ventricular outflow tract obstruction of unclear etiology was also reported on transthoracic echocardiography, necessitating retrieval of the device and surgical closure of the defect. Intraoperative transesophageal echocardiography (TEE) showed right ventricular outflow obstruction by the cocoon device itself with a normal pulmonary valve. In this report, we emphasize that improper device selection for closure of ruptured sinus of Valsalva aneurysm, may lead to delayed leaks across the device, which can gradually progress causing hemolytic anemia and high gradient across the right ventricular outflow tract. Intraoperative TEE helped to delineate the cause of right ventricular outflow tract obstruction. [ABSTRACT FROM AUTHOR]

Marked aneurysmal dilation of the central and branch pulmonary arteries in utero in patients with tetralogy of Fallot with absent pulmonary valve can often exhibit extrinsic compression of the trachea and bronchi. The major morbidity in these patients remains postoperative ventilation issues. This case report highlights the role of intraoperative bronchoscopy in providing guidance for obtaining optimal bronchial decompression that was achieved by an initial pulmonary arteriopexy followed by an aortopexy. [ABSTRACT FROM AUTHOR]

The pulmonary dominant variant of the common arterial trunk has always been reported to be associated with aortic coarctation, or interruption of the aortic arch, along with a duct-dependent systemic circulation. This mandates a complex surgical repair with attendant high surgical mortality. We report a 23-day-old baby with a pulmonary dominant trunk with mild aortic hypoplasia but with an arch free of coarctation or interruption, who underwent successful surgical repair. In the preoperative evaluation of a common arterial trunk, pulmonary dominance may not necessarily denote an adverse risk factor when the aorta is only mildly hypoplastic. [ABSTRACT FROM AUTHOR]

Introduction: Tetralogy of Fallot requiring transannular repair of the right ventricular outflow tract (RVOT) are exposed to free pulmonary insufficiency and hence inevitable right ventricular dysfunction. This study analyzes the function and structure of untreated autologous pericardium monocusp used to create a competent pulmonary valve.Materials and Methods: This is a retrospective analysis of 52 cases operated between December 2006 and December 2012. Untreated autologous pericardium was used for creating a competent pulmonary valve following a transannular patch. They are followed for functional and structural assessment of the pulmonary valve by echocardiography. Positron emission tomography (PET) with 18 fluorodeoxyglucose was performed in two cases for profiling the pulmonary valve.Results: Median age was 10.5 years (1-38). The follow-up was complete for 42 (80.76%) patients for 3 years and 25 (48.07%) patients for 5 years. The RVOT gradient was 42 mmHg (16-96) in the year of surgery, which reduced to 26 mmHg (10-58) and pulmonary insufficiency that was present in 8.3% of patients in 1st year was witnessed in 22.7% in the 5th year of follow-up. The monocusp patch was successful in creating a competent valve while maintaining its structure at 3 years; however, it became distorted and retracted at 5 years of follow-up. There was no calcification in any of the patients. PET-computed tomography confirmed the uptake of glucose by monocusp at 1 year of follow-up.Conclusion: The untreated autologous pericardium functioned well when it was used to create a competent pulmonary valve at short term and midterm. Although it changed in its structure; there was no calcification at 5 years of follow-up. [ABSTRACT FROM AUTHOR]

Background: Complete atrioventricular septal defect (CAVSD) in association with tetralogy of Fallot is a rare and complex disease that makes its repair more difficult than repair of either lesion alone. We reviewed retrospectively our experience in managing this lesion. Patients and Methods: Between February 2006 and May 2017, 16 patients who underwent repair of CAVSD/tetralogy of Fallot (TOF) were reviewed retrospectively. Fifteen patients had trisomy 21. Five patients underwent primary repair while eleven patients went for staged repair in the form of right ventricular outflow tract (RVOT) stenting (n = 9) or systemic to pulmonary (S-P) surgical shunt (n = 2). RVOT stenting has replaced surgical shunt since 2012 in our center. Early presentation with cyanosis was the main determinant factor for staged versus primary repair. Results: The median age at first palliation was 46 days (range 15-99 days). The median age at total repair for both groups was 6 months (range 3-18 months); the median age for the palliated patients was 6.5 months (range 5-18 months) while the median age for primary repaired patients was 5 months (range 3-11 months). The median weight at final repair was 6.9 kg (3.7-8.2 kg). The pulmonary valve was preserved in five patients (31%), four of them had no prior palliation. Chylothorax occurred in 50% of the patients. One late mortality occurred after final repair due to sepsis. Conclusion: CAVSD/TOF can be repaired with low mortality and morbidity. The use of RVOT stent has replaced the surgical (S-P) shunt with acceptable results in our center. [ABSTRACT FROM AUTHOR]

Background: Application of transannular patch (TAP) during the repair of tetralogy of Fallot (TOF) leads to the development of pulmonary regurgitation (PR). This PR is known to cause right ventricular (RV) volume overload and dysfunction which in turn leads to increase in both morbidity and mortality both in immediate and long-term periods. Here, we sought to analyze the effects of polytetrafluoroethylene (PTFE) pulmonary bicuspid valve on the early outcome of patients with TOF repair where TAP is needed. Subjects and Methods: This is a retroprospective, observational study where PTFE bicuspid pulmonary valve was incorporated in all consecutive patients undergoing repair of TOF involving the application of TAP. Postoperative inhospital course was assessed, and patients were followed till 6 months for the status of PR and peak RV outflow tract (RVOT) gradient. Results: 38 patients were enrolled in the study. The mean age of participants was 53.66 ± 78.67 months. Extubation in operating room was done in 31 (81.58%) patients. Duration of ventilator support was 10.48 ± 24.22 h, and duration of hospital stay was 7.95 ± 3.08 days. These data were compared with three recent studies where only TAP was applied. Extubation in operating room, mechanical ventilation time, and hospital stay were significantly improved in the present study. Out of 37 live patients, four (10.81%) patients had no PR, 28 (75%) had mild, 1 (2.7) had moderate, and 4 (10.81%) had severe PR. Average RVOT peak gradient was 18.8 ± 8.1 mmHg. Conclusion: Incorporation of PTFE bicuspid pulmonary valve during the repair of TOF appears to facilitate early extubation and thus reduces ventilator-associated comorbidities. Study needs further long-term follow-up to look for behavior of PTFE bicuspid valve. [ABSTRACT FROM AUTHOR]

Bicuspidity of both the semilunar valves is rarely reported. We report the first ever case of bilateral bicuspid semilunar valves in a case of transposition. [ABSTRACT FROM AUTHOR]

Aortopulmonary window is an uncommon truncal anomaly. Its coexistence with tetralogy of Fallot (TOF) with pulmonary atresia is rare. This rare complex association is always a surgical challenge due to lack of an established surgical approach. The association with TOF makes the interventional approach unlikely. In the two cases summarized below, we have addressed the investigation strategy reaching the final diagnosis. The surgical interventions and our decision-making in the final adopted technique have been highlighted. The relevant surgical outcome with postoperative follow-up has been discussed. The rarity of this association without a standard management algorithm mandates the relevance of this presentation. [ABSTRACT FROM AUTHOR]

Balloon pulmonary valvuloplasty (BPV) is an interventional procedure that has practically replaced the need of open-heart surgery in patients with severe valvular pulmonary stenosis. However, BPV in dysplastic pulmonary valve (PV) still remains a challenge in the current milieu of advanced technologies. We encountered a unique situation of severe restenosis of PV wherein the attempt to balloon dilate the valve was futile because no balloon remained stable at the level of the valve. This situation was overcome using a PDA device delivery sheath to keep the balloon positioned at the desired location. [ABSTRACT FROM AUTHOR]

Transcatheter pulmonary valve replacement using Melody valve (Medtronic, Minneapolis MN) has significantly increased in the recent decades. Melody valve failures, although rare, can be problematic and require re-intervention. Through intracardiac echocardiography, we present two patients who each had a rare etiology for dysfunction of their Melody valve. Hammock effect, wherein the valve does not oppose the stent and complete cusp failure causing severe regurgitation have not been previously described as causes of Melody valve failure in the absence of endocarditis. Awareness and knowledge of these mechanisms is pivotal in the management of this patient population. [ABSTRACT FROM AUTHOR]

Transposition of great arteries (TGA) can be associated with left ventricle outflow tract (LVOT) obstruction. In the presence of ventricular septal defect (VSD), septal leaflet of tricuspid valve may prolapse through perimembranous VSD or rarely tricuspid valve tissue may override to produce LVOT obstruction. Occasionally, this may be mistaken for vegetation due to associated pulmonary valve endocarditis. We report a case of d-TGA with presumptive pulmonary valve endocarditis and LVOT obstruction that was found to be due to tricuspid valve straddling on transesophageal echocardiography, resulting in change in the surgical plan and thus avoiding catastrophe. [ABSTRACT FROM AUTHOR]

Objective : To establish normal reference ranges for cardiac valve cross-sectional areas (CSAs) in preterm infants and their correlation with gestational age, body weight, and chronological age. Materials and Methods : In a prospective study, 268 preterm babies fulflling the criteria for inclusion were examined. Echocardiograms were performed to measure aortic, pulmonary, mitral, and tricuspid valve CSAs on 0-6 day (s) of life and at weekly intervals until they reached 36 weeks. Gestational age was divided into three groups, 24-27, 28-31, and 32-35 weeks, and body weight was divided into fve groups, ≤999, 1000-1499, 1500-1999, 2000-2499, and ≥2500 g. Overall group differences were compared for each period of life: 0-6 days and 1-2, 3-4, and ≥5 weeks. Results : The mean gestational age was 29.8 (±2.38 standard deviation [SD]) weeks, ranging between 24 and 35 weeks, and the mean body weight was 1479 (±413 SD) g, ranging between 588 and 3380 g. All cardiac valve CSAs correlated well with body weight. A signifcant gradual increase was observed in all valve CSAs with body weight during each period of life. Overall, a progressive and signifcant increase in all valve CSAs was observed during the frst 9 weeks of life. Conclusions : Cardiac valve CSAs were found to be signifcantly correlated with body weight. The study also provides reference data, which can be used as a normal reference tool for valve CSAs in preterm infants against gestational age, body weight, and chronological age. [ABSTRACT FROM AUTHOR]

Objective: The study was done to compare propofol and ketofol for sedation of pediatric patients scheduled for elective pulmonary valve implantation in a catheterization laboratory.Design: This was a double-blind randomized study.Setting: This study was conducted in Prince Sultan Cardiac Centre, Saudi Arabia.Patients and Methods: The study included 60 pediatric patients with pulmonary regurge undergoing pulmonary valve implantation.Intervention: The study included sixty patients, classified into two groups (n = 30). Group A: Propofol was administered as a bolus dose (1-2 mg/kg) and then a continuous infusion of 50-100 μg/kg/min titrated as needed. Group B: Ketofol was administered 1-2 mg/kg and then infusion of 20-60 μg/kg/min. The medication was prepared by the nursing staff and given to anesthetist blindly.Measurements: The monitors included heart rate, mean arterial blood pressure, respiratory rate, temperature, SPO2and PaCO2, Michigan Sedation Score, fentanyl dose, antiemetic medications, and Aldrete score.Main Results: The comparison of heart rate, mean arterial pressure, respiratory rate, temperature, SPO2and PaCO2, Michigan Sedation Score, and Aldrete score were insignificant (P > 0.05). The total fentanyl increased in Group A more than Group B (P = 0.045). The required antiemetic drugs increased in Group A patients more than Group B (P = 0.020). The durations of full recovery and in the postanesthesia care unit were longer in Group A than Group B (P = 0.013, P < 0.001, respectively).Conclusion: The use of propofol and ketofol is safe and effective for sedation of pediatric patients undergoing pulmonary valve implantation in a catheterization laboratory. However, ketofol has many advantages more than the propofol. Ketofol has a rapid onset of sedation, a rapid recovery time, decreased incidence of nausea and vomiting and leads to rapid discharge of patients from the postanesthesia care unit. [ABSTRACT FROM AUTHOR]

Background : Surgical and transcatheter bioprosthetic valves (BPVs) in the pulmonary position in patients with congenital heart disease may ultimately fail and undergo transcatheter reintervention. Angiographic assessment of the mechanism of BPV failure has not been previously described. Aims : The aim of this study was to determine the mode of BPV failure (stenosis/regurgitation) requiring transcatheter reintervention and to describe the angiographic characteristics of the failed BPVs and report the types and efficacy of reinterventions. Materials and Methods : This is a retrospective single-center review of consecutive patients who previously underwent pulmonary BPV placement (surgical or transcatheter) and subsequently underwent percutaneous reintervention from 2005 to 2014. Results : Fifty-five patients with surgical (41) and transcutaneous pulmonary valve (TPV) (14) implantation of BPVs underwent 66 catheter reinterventions. The surgically implanted valves underwent fifty reinterventions for indications including 16 for stenosis, seven for regurgitation, and 27 for both, predominantly associated with leaflet immobility, calcification, and thickening. Among TPVs, pulmonary stenosis (PS) was the exclusive failure mode, mainly due to loss of stent integrity (10) and endocarditis (4). Following reintervention, there was a reduction of right ventricular outflow tract gradient from 43 ± 16 mmHg to 16 ± 10 mmHg (P < 0.001) and RVp/AO ratio from 0.8 ± 0.2 to 0.5 ± 0.2 (P < 0.001). Reintervention with TPV placement was performed in 45 (82%) patients (34 surgical, 11 transcatheter) with no significant postintervention regurgitation or paravalvular leak. Conclusion : Failing surgically implanted BPVs demonstrate leaflet calcification, thickness, and immobility leading to PS and/or regurgitation while the mechanism of TPV failure in the short- to mid-term is stenosis, mainly from loss of stent integrity. This can be effectively treated with a catheter-based approach, predominantly with the valve-in-valve technique. [ABSTRACT FROM AUTHOR]

Background : Perforation of pulmonary valve using radiofrequency ablation in pulmonary atresia with intact ventricular septum (PA IVS) is a treatment of choice. However, significant cost of the equipment limits its utility, especially in the developing economies. Objective : To assess the feasibility, safety, and efficacy of perforation of pulmonary valve using chronic total occlusion (CTO) wires in patients with PA IVS as an alternative to radiofrequency ablation. Methods : This is a single-center, nonrandomized, retrospective study conducted during June 2008 to September 2015. Twenty-four patients with PA IVS were selected for the procedure during the study period. The median age and weight of the study population were 8 days and 2.65 kg, respectively. Four patients were excluded after right ventricular angiogram as they showed right ventricular-dependent coronary circulation. The pulmonary valve perforation was attempted using various types of CTO wires based on the tip load with variable penetrating characteristics. Results : The procedure was successful in 16 of twenty patients using CTO wires: Shinobi in nine, Miracle in four, CROSS-IT in two, and Conquest Pro in one. Two patients had perforation of right ventricular outflow tract (RVOT). Pericardiocentesis was required in one patient to relieve cardiac tamponade. Later, the same patient underwent successful hybrid pulmonary valvotomy. The other patient underwent ductus arteriosus (DA) stenting. Balloon atrial septostomy was needed in three cases with systemic venous congestion. Desaturation was persistent in five cases necessitating DA or RVOT stenting to augment pulmonary blood flow. There were two early and two late deaths. The mean follow-up was 22.66 ± 16 months. Three patients underwent one and half ventricle repair and one Blalock-Taussig shunt during follow-up. Conclusion : Perforation of the pulmonary valve can be done successfully using CTO wires in selected cases of pulmonary atresia with intact ventricular septum. [ABSTRACT FROM AUTHOR]

Perioperative management of a patient with Dandy-Walker malformation (DWM) with tetralogy of Fallot (TOF), patent ductus arteriosus, and pulmonary artery stenosis is a great challenge to the anesthesiologist. Anesthetic management in such patients can trigger tet spells that might rapidly increase intracranial pressure (ICP), conning and even death. The increase in ICP can precipitate tet spells and further brain hypoxia. To avoid an increase in ICP during TOF corrective surgery ventriculo-peritoneal (VP) shunt should be performed before cardiac surgery. We present the first case report of a 11-month-old male baby afflicted with DWM and TOF who underwent successful TOF total corrective surgery and fresh autologous pericardial pulmonary valve conduit implantation under cardiopulmonary bypass after 1 week of VP shunt insertion. [ABSTRACT FROM AUTHOR]

Background and Objectives: Repair of tetralogy of Fallot (TOF) with monocusp pulmonary valve reconstruction prevents pulmonary regurgitation (PR) for a variable period. Since postoperative outcome is governed by PR and right ventricular function, we sought to assess the severity of pulmonary regurgitation and right ventricular outflow (RVOT) gradient in the immediate postoperative period and at 1 year and attempted to identify the anatomical substrates responsible for adverse outcomes. Methods : The study included 30 patients. Transthoracic echocardiography was performed before surgery, within 5 days of surgery, and 1 year later. Presence and severity of PR, RVOT gradient, and residual branch pulmonary stenosis were assessed. Right ventricular and monocusp valve functions were studied. Results : Median age was 36.5 months (3-444 months). There were no deaths. Pulmonary regurgitation was mild in 18, moderate in 10, and severe in 2 patients immediately following surgery. At 1 year, 10 patients had severe PR and one had significant RVOT gradient. None of the variables like age, presence of supravalvar pulmonary branch stenosis, main pulmonary artery diameter, or mobility of monocusp valve was found to have any significant association with the progression of PR. McGoon index <1.5 showed a trend toward more PR, while patients with more residual RVOT gradient had lesser regurgitation. Conclusions : Repair of TOF with monocusp pulmonary valve reduces immediate postoperative PR. At 1 year, the monocusp valve underwent loss of function in a significant proportion and PR also progressed. This study could not identify any predictors of progression of PR, though patients with McGoon index <1.5 tended to have more PR while those with more outflow gradient had lesser PR. [ABSTRACT FROM AUTHOR]

Functional pulmonary atresia is characterized by a structurally normal pulmonary valve that does not open during right ventricular ejection. It is usually associated with Ebstein's anomaly, Uhl's anomaly, neonatal Marfan syndrome and tricuspid valve dysplasia. However, functional pulmonary atresia is rarely reported in newborn with anatomically normal heart. We report a newborn with functional pulmonary atresia who had normal intracardiac anatomy, who responded to treatment with nitric oxide and other vasodilator therapy successfully. [ABSTRACT FROM AUTHOR]

The article discusses percutaneous pulmonary valve implantation with the Melody valve by Medtronic as indicated for regurgitant lesions. It outlines the patho-physiologhical criteria for selecting patients for implantation and discusses preprocedure assessment of patients. It also describes the procedure in detail, its intra and post operative complications and management.

Objective: The bidirectional Glenn shunt is commonly performed under cardiopulmonary bypass for conditions that lead to a single ventricle repair. We report our experience of bidirectional Glenn shunt done without cardiopulmonary bypass. Methods: Between June 2007 and May 2009, 186 consecutive patients underwent off-pump bidirectional Glenn shunt for a variety of complex cyanotic congenital heart defects. Age ranged from four months to six years and the median weight was 11.17 kg (range 4.3 - 18). After systemic heparinization, the procedure was done by creating a temporary shunt between the innominate vein and the right atrium connected across a three way connector for de-airing. Fifty one patients had bilateral cavae. All cases underwent complete clinical neurological examination. Results: No case required conversion onto cardiopulmonary bypass. Four patients (2.14%) died in the immediate postoperative period. The mean internal jugular venous pressure on clamping the decompressed superior vena cava was 24.69 ± 1.81 mm Hg. There was no intra-operative hemodynamic instability and oxygen saturation was maintained at more than 70% throughout. Post Glenn shunt, the saturations improved to mid 80s. Seventy four cases had documented forward flow across the pulmonary valve. The mean duration of ventilation was 10.17 ± 8.96 hours and there were no neurological complications. Six patients (3.22%) developed pleural effusions, 4 patients (2.15%) had nodal rhythm and 9 patients (4.83%) had superficial sternal wound infection. Conclusions: Our results show that off-pump bidirectional Glenn shunt can be done safely in patients not requiring associated intra-cardiac correction. It avoids cardiopulmonary bypass and its related complications, is economical and associated with excellent results. In our opinion, this is the largest series of off-pump bidirectional Glenn shunt in the literature. [ABSTRACT FROM AUTHOR]

Transesophageal echocardiography has been shown to provide unique information about cardiac anatomy, function, hemodynamics and blood flow and is relatively easy to perform with a low risk of complications. Echocardiographic evaluation of the tricuspid and pulmonary valves can be achieved with two-dimensional and Doppler imaging. Transesophageal echocardiography of these valves is more challenging because of their complex structure and their relative distance from the esophagus. Two-dimensional echocardiography allows an accurate visualization of the cardiac chambers and valves and their motion during the cardiac cycle. Doppler echocardiography is the most commonly used diagnostic technique for detecting and evaluating valvular regurgitation. The lack of good quality evidence makes it difficult to recommend a validated quantitative approach but expert consensus recommends a clinically useful qualitative approach. This review enumerates probe placement, recommended cross-sectional views, flow patterns, quantitative equations including the clinical approach to the noninvasive quantification of both stenotic and regurgitant lesions. [ABSTRACT FROM AUTHOR]

Endocarditis of percutaneously placed pulmonary valve is increasingly being recognized and reported as a potentially life-threatening complication. In this report, we discuss a 17-year-old male who presented with septic shock secondary to staphylococcal endocarditis of a percutaneously placed pulmonary valve. [ABSTRACT FROM AUTHOR]

A 62-year-old obese woman with a history of atypical chest pain and cardiac murmurs was referred to our echocardiography laboratory for further evaluations. Despite poor echocardiography windows, transthoracic echocardiography revealed that the base and mid segments of the anteroseptal and inferoseptal walls were hypertrophied with a maximal thickness of 15 mm and a moderate-to-severe subaortic stenosis with a peak pressure gradient of 57 mmHg. Transesophageal echocardiography showed no aortic valve stenosis, no mitral valve prolapse, trivial mitral regurgitation, and a significant systolic anterior motion of the mitral chordae tendineae [[Figure 1] and Videos 1-4]. [Extracted from the article]

We report a newborn with an unusual combination of aneurysmally dilated thin-walled right ventricle with hypertrophy of the apical muscles of the right ventricle. There was narrow pulmonary annulus, pulmonary regurgitation, and hypoplasia of the mitral valve and left ventricle. We propose that this heart represents a partial form of Uhl&grave;s anomaly. [ABSTRACT FROM AUTHOR]

Stenosis of systemic semilunar valve in cyanotic congenital heart defects is rare. It can happen in truncus arteriosus with truncal valve stenosis and the very rare anomaly of tetralogy of fallot with aortic valve stenosis. Here we describe a neonate with pulmonary atresia, ventricular septal defect and associated aortic valve stenosis and discuss the points of differentiation from truncus arteriosus. [ABSTRACT FROM AUTHOR]

Transesophageal echocardiography can be a useful adjunct in assessing the quality of repair in patients undergoing novel methods of reconstruction of the right ventricular outflow. We present one such patient here. [ABSTRACT FROM AUTHOR]

A letter to the editor is presented which focuses on the future of Ross procedure which has been at the center of a scientific debate, along with issues associated to the procedure such as pulmonary autograft (PA) dilation and potential for reoperation.