Early and Long-Term Clinical Outcomes of Ductal Stenting Versus Surgical Aortopulmonary Shunt Among Young Infants with Duct-Dependent Pulmonary Circulation.

Surgical aortopulmonary shunting (SAPS) and ductal stenting (DS) are the main palliations in infants with cyanotic congenital heart diseases (CHD). We aimed to study the safety and efficacy of DS and to compare it with SAPS as a palliative procedure in infants with CHD and duct-dependent pulmonary circulation. Retrospective institutional clinical data review of consecutive infants aged < 3 months who underwent DS or SAPS over 5 years. The primary outcome was procedural success which was defined as event-free survival (mortality, need for re-intervention, procedural failure) at 30 days post-procedure. The secondary outcome was defined by a composite of death, major adverse cardiovascular events, or need for re-intervention at 6 months and on long-term follow-up. We included 102 infants (DS, n = 53 and SAPS, n = 49). The median age at DS and SAPS was 4 days (IQR 2.0-8.5) and 8 days (IQR 4.0-39.0), respectively. The median weight at intervention was 3.0 kg (IQR 3.0-3.0) and 3.0 kg (IQR 2.5-3.0) in the two respective arms. Tetralogy of Fallot with pulmonary atresia was the most common indication for DS and SAPS. The 30-day mortality was significantly higher in SAPS group as compared with DS group (p < 0.05). However, 30-day major adverse cardiac events (MACE) rates were similar in both groups (p = 0.29). DS was associated with shorter duration of mechanical ventilation, duration of stay in the intensive care and hospital stay than with SAPS. At 6 months, there was no significant difference in terms of mortality or event-free survival. Long-term MACE-free survival was also comparable (p = 0.13). DS is an effective and safer alternative to SAPS in infants with duct-dependent pulmonary circulation, offering reduced procedure-related mortality and morbidity than SAPS. Careful study of ductal anatomy is crucial to procedural success. However, long-term outcomes are similar in both procedures.

Clinical profile and outcomes of childhood dilated cardiomyopathy - A single-center three-decade experience.

Introduction and Aims: Dilated cardiomyopathy (DCM) is an important cause of heart failure (HF) among children. Research on pediatric DCM remains surprisingly scarce. The primary objective of the study was to evaluate the clinical profile and outcomes of pediatric DCM and the secondary objective was to study the predictors of outcome. Methods and Results: We enrolled all patients with cardiomyopathy who presented to us between 1990 and 2020 and were younger than 18 years. During the 30-year study period, we identified 233 cases of pediatric cardiomyopathy. One hundred and nineteen (51%) cases had DCM. This retrospective cohort was analyzed to study their outcome and the possible predictors of outcome. Nearly, 8% presented in the neonatal period, and 37% in infancy. The most common mode of presentation was dyspnea on exertion (71%). Ninety-three patients presented in heart failure (78%). The median left ventricular dimension z-score in diastole was 4.3 (range 2.5-9.06). The median left ventricle (LV) ejection fraction was 31%. Seventy-two percent of this cohort were on angiotensin-converting-enzyme inhibitors, 40% on aldosterone antagonists, and 47% on beta-blockers. One-third had syndromic, metabolic, genetic, or any secondary cause identified. Twenty-seven patients satisfied the three-tiered clinical classification for the diagnosis of probable acute myocarditis. Over a mean follow-up of 3.29 years, 27% were lost to follow-up. Among the remaining patients who were on follow-up (n = 86), 39 (45%) died, 31 (36%) recovered, and 16 (18%) had persistent LV dysfunction. Heart Failure was the most common cause of death. Eight patients in this cohort (4.2%) had thromboembolic phenomena. Nine had sustained ventricular arrhythmias and six had atrial/junctional arrhythmias. Among the various risk factors studied, only infantile onset had a significant relationship with death or ventricular arrhythmias (P value- 0.05). The 5-year survival rate of DCM patients was 59%. Conclusion: A reasonably good percentage of our population showed recovery of the left ventricular function (36%). Only infantile onset had a significant relationship with death or ventricular arrhythmias. The outcome in our DCM cohort is similar to other population cohorts.

Pancycle aortic incompetence and double alternans in pediatric heart failure.

A 12-year-old boy presented with bicuspid aortic valve, severe aortic regurgitation, and dilated dysfunctional left ventricle in heart failure. He underwent aortic valve replacement with a 23 mm TTK Chitra heart valve prosthesis (tilting disk). He was gradually weaned off milrinone and noradrenaline in the intensive care. Echocardiography showed severe left ventricular dysfunction with an ejection fraction of 24%. The radial pulse was regular and of normal volume but exactly half that of the heart rate. Evaluation of the rhythm and echocardiography revealed an interesting hemodynamic phenomenon with double alternans.

Clinical profile and outcomes of pediatric hypertrophic cardiomyopathy in a South Indian tertiary care cardiac center: a three decade experience.

INTRODUCTION: Although much research has been done on adult hypertrophic cardiomyopathy, data on pediatric hypertrophic cardiomyopathy is still limited. METHODS AND RESULTS: The study enrolled all patients with cardiomyopathy who presented to us between 1990 to 2020 and were younger than 18 yrs. During the thirty-year study period, we identified 233 cases of pediatric cardiomyopathy. Sixty-three cases (27%) had hypertrophic cardiomyopathy. Out of the 63 HCM cases, 12% presented in the neonatal period and 37% presented in the first year of life. The median age of presentation was 7 yrs (Range 0.1-18 yrs). Sixteen patients had proven syndromic, metabolic, or genetic disease (25%). LV outflow obstruction was present in 30 patients (47%). Noonan syndrome was present in 9 of the 63 patients (14%). Dyspnea on exertion was the most common mode of presentation. Cardiac MRI was done in 28 patients, out of which 17 had late gadolinium enhancement (LGE). Mid myocardial enhancement was the most common pattern. Four patients had LGE of more than 15%. Over a mean follow-up period of 5.6 years (0.1-30 years), twenty-one were lost to follow-up (33%). Among the patients whose outcome was known, eleven died (26%), and thirty-one (73%) were alive. The 5-year survival rate of HCM patients was 82%, and the 10-year survival rate was 78%. Seven died of sudden cardiac death, three from heart failure, and one from ventricular arrhythmias. Sustained ventricular arrhythmias were seen in three patients and atrial arrhythmias in two. First-degree AV block was seen in 10 patients (15%) and bundle branch blocks (BBB) in five (8%). Eight patients required ICD or transplant (12.7%). Two patients underwent ICD for primary prevention, and one underwent PPI for distal AV conduction disease. Among the various clinical, echocardiographic, and radiological risk factors studied, only consanguinity showed a trend towards higher events of death or ventricular arrhythmias (P-value 0.08). CONCLUSION: More than one-third of our HCM cohort presented in infancy. LV outflow tract obstruction is common (47%). Mid myocardial enhancement was the most common pattern of late gadolinium enhancement. SCD was the most common cause of death. The outcome in our HCM cohort is good and similar to other population cohorts. Only Consanguinity showed a trend towards higher events of death or ventricular arrhythmias.

Postoperative Aortic Regurgitation in Outflow Ventricular Septal Defect: Determinants of Outcome.

Aortic cusp prolapse is an acquired complication and usually precedes the development of aortic regurgitation (AR) in unoperated outflow ventricular septal defect (VSD). However, its impact on postoperative AR-progression is unknown. 161 patients with outflow-VSD and AR who underwent surgery between 2006 and 2012 were studied retrospectively. 31 patients without prolapse (group-I), 87 with only right coronary cusp (RCC) (group-II), 43 with noncoronary cusp (NCC) prolapse (group-III: 23 only NCC (IIIa), 20 both NCC-RCC (IIIb)) were followed postoperatively for a mean 6.05 ± 2.4 years (range 3-12 years). Moderate or severe-AR was present in 4.2%, 36.8%, 52.2% and 80% preoperatively; in 3.2%, 10.3%, 39.1% and 30% patients at follow-up in group-I, II, IIIa, and IIIb, respectively. Although freedom from significant-AR (moderate or severe AR) or aortic valve replacement (AVR) at 10 years was lesser in subaortic-VSD than subpulmonic-VSD (64.3 ± 7.5% vs 87.9 ± 3.6%; P = 0.02), the difference was not significant when compared within prolapse groups (80 ± 8% vs 88.7 ± 4.0%, P = 0.28 in group-II; 40.7 ± 11.8 vs 70 ± 14.5%, P = 0.48 in group-III). The significant-AR or AVR free survival in patients with trivial or mild preoperative-AR was not significantly different between prolapse groups (98.2 ± 1.8% vs 75 ± 21.7% in group-II and III respectively; P = 0.85). However, in those with moderate or severe preoperative-AR it was significantly lesser in group-III than II (30.1 ± 9.8% vs 65.6 ± 8.4%, respectively; P = 0.04). Group-III, compare to group-II, had 3.28 and 5.24-time risk of development of significant-AR or requirement of AVR, respectively. Prolapse of NCC alone or in addition to RCC prolapse has unfavourable impact on the postoperative outcomes, especially in subaortic-VSD after development of more than mild AR preoperatively.

Outflow Ventricular Septal Defect With Aortic Regurgitation: Optimal Timing of Surgery?

BACKGROUND: Ideal time of surgery still remains controversial in outflow ventricular septal defect (VSD) with aortic regurgitation (AR). We aimed to identify the prevalence and predictors of postoperative AR progression. METHODS: A total of 154 patients with outflow VSD and AR who underwent VSD surgery between 2006 and 2012 were studied retrospectively. RESULTS: Eighty patients with subpulmonic VSD and 74 with subaortic VSD were followed up for mean 6.32 ± 2.27 years (range, 3-12 years). Of these, 100 had trivial to mild (group A) and 54 had moderate to severe preoperative AR (group B). At follow-up, there was no significant worsening of mean residual AR grade in group A (P = .16) and subpulmonic VSD of group B (P = .083). However, AR grade worsened significantly in subaortic VSD (1.85 ± 0.87 vs 2.21 ± 1.08, P = .005) of group B. Only 2 (both had subaortic VSD) patients of group A developed moderate AR and none required aortic valve replacement (AVR), while 23 (42.60%) of group B patients developed moderate or severe AR and 7 (30.4%) of them required AVR. Moreover, all who needed AVR had subaortic VSD and had undergone valvuloplasty during VSD closure. The 10 years freedom from moderate or severe AR was significantly lower in group B than group A in both VSDs (subaortic VSD 42.5% ± 10.7% vs 89.3% ± 8.1%, P < .01; subpulmonic VSD 66.7% ± 10.3% vs 100%, P< .01). On multiple regression analysis, postoperative residual AR was the only predictor of AR progression (standardized coefficient, 0.48; P < .001) at follow-up. CONCLUSIONS: Mild preoperative AR rarely progressed after VSD repair. However, worsening of AR could not be prevented effectively, even with valvuloplasty, after the development of moderate or severe AR. Mild or more postoperative residual AR requires close follow-up, especially in subaortic VSD.

A Comparative Study of Invasive Modalities for Evaluation of Pulmonary Arteriovenous Fistula after Bidirectional Glenn Shunt.

Development of pulmonary AV fistula (PAVF) after bidirectional glenn shunt (BDG) results in significant cyanosis, impaired exercise performance, and increased morbidity and mortality. We attempted to detect and quantify PAVF in post-BDG patients by saline contrast transesophageal echocardiography (TEE) and compare with pulmonary angiography and pulmonary vein oximetry. This was a prospective study done between 2017 and 2018. Twenty-five children who underwent BDG and planned for cardiac catheterization prior to Fontan completion were included in the study. All patients underwent pulmonary angiography, oximetry, and saline contrast TEE at the time of cardiac catheterization. Twenty-two patients had undergone unilateral BDG surgery and three were palliated by bilateral BDG. The mean oxygen saturation was 80 ± 5.2%. Thirteen patients (52%) had preserved antegrade pulmonary blood flow. Eighteen patients (72%) had PAVF by angiography and oximetry, while 19 (76%) had PAVF identified by contrast echocardiography. There was moderate correlation between the degree of pulmonary venous desaturation and grading of PAVF by contrast echocardiography. PAVF was predominantly located in the lower zones of the lungs. Higher grades of PAVF were not seen in patients with preserved antegrade flow after BDG. Angiographically detected PAVF showed a steady increase with increasing delay to cardiac catheterization from BDG. Significant reduction in systemic saturation was limited to advanced grades of PAVF in patients after BDG. Saline contrast TEE, pulmonary venous oximetry, and pulmonary angiography equally identified PAVF in patients after BDG. Prognostic utility of the same needs to be assessed by long-term follow-up of these subjects.

Technical considerations of coarctation stenting in double-barreled aorta - A persistent fifth arch mimic.

The double-barreled aorta connecting the ascending aorta and descending aorta caudal to the normal fourth aortic arch has fascinated the interests of cardiac morphologists for over a century. This condition is commonly associated with coarctation. While the controversies surrounding the embryology of the double-barreled aorta have settled down, we present a case-based illustration of the technical aspects of coarctation stenting peculiar to this condition.

Clinical profile & long-term natural history of symptomatic coronary artery disease in young patients (<30 yr).

Background & objectives: Several studies have shown a high prevalence of cardiovascular risk factors in patients in the age group of 30-74 yr, but there is a paucity of data in young patients below 30 yr. We analyzed the clinical and coronary angiographic profile of patients <30 yr of age with symptomatic coronary artery disease (CAD) and also assessed their intermediate and long-term outcomes. Methods: All patients less than 30 yr of age who presented with symptomatic CAD from 1978 to 2017 in the department of Cardiology of a tertiary care hospital in south India, were studied for coronary risk factors and angiographic and treatment patterns, and the follow up data were collected. Results: The mean age of the 159 patients <30 yr of age was 26.7±3.29 yr. Male preponderance was observed (91.8%), 63.5 per cent patients were smokers and 88.3 per cent were dyslipidaemic. Acute myocardial infarction was the most common mode of presentation. Forty one per cent patients were non-adherent to medications. Risk factor control was inadequate with respect to smoking cessation, alcoholism, physical activity and dietary regulation. The predictors of long-term mortality were multivessel CAD [hazard ratio (HR): 1.927, 95% confidence interval (CI): 1.003-3.701] and reduction in ejection fraction (EF) (10% decrease in EF; HR: 1.349, 95% CI: 1.096-1.662). Overall mortality was 30 per cent at 10 yr and 48 per cent at 20 yr. Interpretation & conclusions: Decreasing EF and multivessel involvement were found to be the strong correlates for long-term mortality in young patients below 30 yr of age with CAD. High long-term mortality rates and poor risk factor control suggest the vast scope for the improvement of outcomes in these patients with aggressive risk factor control.

Early and late outcome of covered and non-covered stents in the treatment of coarctation of aorta- A single centre experience.

OBJECTIVE: Stenting of coarctation of aorta with covered or uncovered stents is the accepted modality of treatment in older children and adults. The indications which mandate the use of covered stents are still unclear. We attempted to study the early and late outcomes after stenting of native and recurrent coarctation of aorta with uncovered and covered stents. METHOD: This is a retrospective study of patients who underwent stenting for coarctation of aorta with covered or non-covered stents at our institute. Early and late outcome for both the groups were studied. RESULTS: Twenty patients underwent implantation of covered stent and twenty five patients had uncovered stent implantation. Patients in the covered stent group were older and had greater basal pressure gradient. More patients in the covered stent group had residual gradient >10 mm Hg after the procedure. There was no mortality or aortic wall injury in either group. Four patients in the covered stent group underwent planned re-intervention and two had unplanned re-intervention. None of the patients in the uncovered stent group had re-intervention. Higher incidence of late lumen loss was noted in the covered stent group. CONCLUSION: Uncovered stents can be safely implanted with minimal risk of aortic wall injury in patients with low risk anatomic features. Covered stent implantation is associated with higher incidence of planned and unplanned re-intervention.

Venous flow patterns after percutaneous atrial septal defect closure: Does the mechanical device decrease right atrial compliance?

INTRODUCTION: Systemic venous flow patterns become abnormal and restrictive after surgical closure of ostium secundum atrial septal defect (ASD) but rarely studied after percutaneous device closure. METHODS: From January 2017 to January 2018, systemic venous Doppler flow patterns were documented prospectively in 50 subjects who underwent percutaneous closure of ASD, prior to, after procedure, and at 6-month follow-up and correlated with defect size and device size. RESULTS: In hepatic veins and superior venacava post device-closure closure, the velocity time integral (VTI) of forward flow in both systole (S) and diastole (D) increased. Overall S was higher than D, and D/S ratio was <1. The D/S ratio increased after device closure significantly reflecting that the improvement in atrial filling increase in diastolic flow more than the increase in systolic flow. Increase in flow velocities was more prominent at 6 months with further increase in D/S VTI ratios. When correlated with the defect size, in those with defect size less than 15 mm/sq.m (mean device size 13.05 ± 3.21 mm), the changes in S- or D-wave, D/S ratio were less prominent and statistically not significant, while in subjects with defect size ≥ 15 mm/sq.m (mean device size 23.02 (±4.77 mm), these changes were greater and statistical significant. CONCLUSION: Residual filling defects with restriction of systolic venous flow were observed in subjects after device closure, correlating with larger device sizes, implying the compliance abnormality conferred by them which progresses at 6 months. Subjects with persistent abnormalities would need careful follow up for incomplete remodeling and increase in atrial size related arrhythmias.

Valentine on a crab-Type A interrupted aortic arch with type 2 aortopulmonary window.

A 10-day-old infant was evaluated for heart failure and differential cyanosis. Type A interrupted aortic arch with duct-dependent lower body circulation was identified. There was associated type 2 aortopulmonary window which led to a "Valentine on a crab" appearance on echocardiography. Pattern recognition in imaging is useful for early identification of anomalies and for triaging appropriate evaluation and management.

Balloon atrial septostomy at the bedside versus the catheterisation laboratory.

A shorter umbilical venous approach provides an opportunity for balloon atrial septostomy in the younger neonate as opposed to those who present at the end of first week of life. However, the ideal choice of access for a bedside balloon atrial septostomy is not well established. Wouldn't prostaglandin infusion be a safer option for transport of babies with dextro-transposition in the neonatal period, when the arterial duct can be kept open? A prenatal diagnosis of dextro-transposition facilitates monitoring and planning of septostomy in the early neonatal period explaining why babies underwent bedside procedures more often.

The mysterious "double" arch.

A child with cyanosis and heart failure was noted to have two different vascular arches of similar caliber on echocardiography. A systematic analysis of the rhythm and flow patterns revealed the mysterious "double" arch.

Role of Diastology in Transcatheter Aortic Valve Implantation.

Transient elevation of LV diastolic pressure above LA pressure can occur in severe aortic regurgitation in the diastasis phase. Mitral E wave reversal in such a situation could serve as non-invasive evidence of elevated left ventricular diastolic pressures, thereby guiding therapeutic decision making.