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.

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.

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.

Left ventricular regression after balloon atrial septostomy in d-transposition of the great arteries.

OBJECTIVES: Balloon atrial septostomy (BAS) is an effective palliative procedure in children with transposition of the great arteries and poor intercirculatory mixing. While the subpulmonary left ventricle (LV) is known to regress with time in these newborns due to the declining afterload, it has not been studied how it behaves following BAS and a consequent decrease in preload. The study was designed to examine the effects of BAS on the LV in infants with simple d-transposition of the great arteries by serial 2D echocardiographic monitoring. METHODS: This was a prospective echocardiographic follow-up study of all consecutive children with simple d-transposition of the great arteries who underwent BAS for restrictive interatrial communication and oxygen saturation below 75% between January 2014 and June 2015. Left ventricular mass estimation was done by M-mode transthoracic echocardiography before balloon septostomy and serially on follow-up till surgery. RESULTS: The median age of the 25 children studied was 4 days (1-95 days) when they underwent BAS. Twenty patients underwent arterial switch operation at a mean of 9 days from balloon septostomy. The mean baseline left ventricular mass was 47.9 g/m2, which decreased to 38.5, 36.2, 32.1, 32.4, 25.7 and 25.2 g/m2 on Days 1, 3, 6, 9, 12 and 15, respectively. The left ventricular mass decreased by 1.5 g/m2 every day during the first 2 weeks following balloon septostomy adjusted for the age of the child in days. Children who underwent BAS beyond 3 weeks of life had faster LV regression than those who underwent the procedure earlier (unstandardized regression coefficient ß 0.892, P < 0.001). CONCLUSIONS: BAS is associated with accelerated regression of the LV in infants with simple d-transposition of the great arteries in the first 2 weeks after the procedure. Regression of the LV is faster in children who underwent BAS after 3 weeks of age.

Asymptomatic right ventricular dysfunction in surgically repaired adult tetralogy of fallot patients.

BACKGROUND: Right ventricular (RV) dysfunction after surgical repair of Tetralogy of Fallot (TOF) is often asymptomatic and may be detected by tissue Doppler imaging (TDI). The severity of RV dysfunction is more after intracardiac repair with transannular patch (TAP). METHODS: One hundred seventy-three adult patients who have undergone surgical repair for TOF were prospectively analyzed for RV function using 2D echocardiography and TDI. RV function was compared between patients who have undergone intracardiac repair with and without TAP. RESULTS: In both the patient sub-groups, TDI derived myocardial performance index (MPI) and myocardial velocities were abnormal even when 2D echocardiography derived RV functional area change was normal. TDI derived MPI was significantly higher (0.5 ± 0.1 vs. 0.4 ± 0 P < 0.001) and Systolic tricuspid annular velocity (Sa) (9.2 ± 1.3 vs. 10.8 ± 1.6 P < 0.001) was significantly lower in the TAP group. Older age at surgery and severity of pulmonary regurgitation on follow-up were among the significant predictors of TDI derived MPI. CONCLUSIONS: Asymptomatic RV dysfunction in surgically repaired adult TOF atients can be detected by TDI. Extent of RV dysfunction was significantly greater with patients requiring TAP, in those operated at older age, and in patients with severe pulmonary regurgitation.

Ventricular septal rupture following myocardial infarction. Long-term survival of patients who did not undergo surgery. Single-centre experience.

Long-term survival is rare in patients not undergoing surgery after post-myocardial infarction ventricular septal rupture. We report our experience of seven patients out of 27, who did not undergo surgery and were followed up for a mean period of 2.8 years. They were evaluated after a mean period of 2.2 months after infarction in our centre. The septal defects measured 9.8 mms on average and the mean left-to-right shunt ratio was 1.98: 1. The mean pulmonary artery, right atrial and left ventricular end diastolic pressures were 28.3 +/- 10.6, 4 +/- 3 and 15.8 +/- 4.8 mm Hg, respectively. Only three out of seven patients had LV aneurysm and all patients had single-vessel disease. Smaller defect size, minimal left-to-right shunt and preserved right ventricular function may be the factors responsible for long-term survival.