Influence of age at surgery on left ventricular strain in patients with anomalous origin of left coronary artery from pulmonary artery.

OBJECTIVES: Myocardial strain abnormalities are described after surgical repair of anomalous left coronary artery from pulmonary artery (ALCAPA) even after recovery of ventricular function. The factors that predispose to the presence of these strain abnormalities in the presence of normal ventricular function are unknown. The aim of this study was to find out whether the age at repair influences the presence of global and regional strain abnormalities on follow-up. METHODS: Repaired ALCAPA patients from a single centre (n = 40) with good ventricular ejection fraction on follow-up were recruited. Baseline and follow-up data were collected from electronic records. Global and regional myocardial strain assessment was done by speckle tracking echocardiography prospectively. The association between age at repair and strain abnormalities on follow-up was analysed. RESULTS: The patients who presented earlier had significantly worse ventricular function pre-operatively compared to older patients (P < 0.0005). Global longitudinal strain was abnormal in 40% of patients with normal ventricular ejection fraction on follow-up. Presence of longitudinal strain abnormalities was more in patients who underwent repair at older age than in those who were repaired earlier (P < 0.0005). The probability of having normal longitudinal strain on follow-up was 81.6% if surgery was done before 7.8 months of age. If operated before 6 months, the odds of having normal myocardial strain was 11 times higher. Regional strain abnormalities of varying severity were present in all patients in the left and in some patients in the right coronary artery territories. CONCLUSIONS: Older age at ALCAPA repair is associated with increased incidence of myocardial strain abnormalities. Regional strain abnormalities were found in both left and right coronary artery territories.

Recurrent pericardial effusion in a boy: A clue to underlying pericardial and pulmonary lymphangiectasia.

Pulmonary lymphangiectasia is a rare congenital malformation of lymphatic vessels. We report the case of a 5-year-old boy with recurrent pericardial effusion which was diagnosed to be due to pericardial and pulmonary lymphangiectasia.

Novel transcatheter treatment for staged closure of Abernethy malformation with portal hypoplasia.

Portosystemic shunts are rare congenital malformations that come to attention due to various hepatic and extrahepatic manifestations. Management of this malformation is dictated by the presence and adequacy of intrahepatic portal radicals. Staged occlusion of the shunt is recommended in patients with severely hypoplastic portal radicals. We describe a novel transcatheter technique that we improvised for staged occlusion of Abernethy malformation in a 2-year-old girl.

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.

Coronary-to-pulmonary artery collaterals in pulmonary atresia.

A 2-month-old baby with ventricular septal defect and pulmonary atresia was found to have coronary-to-pulmonary artery collaterals. Cardiac computed tomography confirmed the coronary collaterals and showed the absence of other systemic to pulmonary artery collaterals. Although these collaterals do not cause coronary ischemia, it is important to delineate them by accurate imaging to plan the appropriate surgical strategy.

The outcome of mitral regurgitation after the repair of anomalous left coronary artery from the pulmonary artery in infants and older children.

OBJECTIVES: Mitral regurgitation (MR) in the majority of infants with an anomalous left coronary artery from the pulmonary artery (ALCAPA) has been shown to improve without concomitant mitral valve repair. However, the outcome of MR in older children with ALCAPA is unclear. The purpose of this study was to compare the outcome of MR following the ALCAPA repair in infants and older children. METHODS: Forty-six patients (34 were younger than 1 year and 12 were aged 1-12 years) underwent the repair for ALCAPA in our institution from June 2006 to June 2016. Baseline and follow-up data were collected from records, and the latest echocardiogram was performed for all surviving patients from June 2017 to August 2017. RESULTS: Of the 34 infants, 82% had moderate-to-severe ventricular dysfunction, and 47% had significant MR at presentation. Fifty-seven percent of the older age group had MR, though none of them had ventricular dysfunction. Two infants and 3 older children underwent concomitant mitral valve repair. There were 2 early deaths in the infantile group. On follow-up, ventricular function normalized in 88% of infants; 12.5% of the infants had moderate-to-severe MR on follow-up, and 50% of the older children had moderate-to-severe MR on follow-up. CONCLUSIONS: The ALCAPA repair has excellent survival outcomes in all age groups. MR improves in the majority of infants with revascularization alone. MR worsens or persists in a significant number of older children following the ALCAPA repair, and they are more likely to require reinterventions for the mitral valve on follow-up.

Quantification of ventricular unloading by 3D echocardiography in single ventricle of left ventricular morphology following superior cavo-pulmonary anastomosis and Fontan completion - a feasibility study.

BACKGROUND: Three-dimensional echocardiography. (3DE) is comparable to cardiac magnetic resonance imaging for estimating ventricular volume in congenital heart diseases. However, there are limited data on estimation of ventricular volumes by 3DE in univentricular heart and change in ventricular volumes after surgical creation of cavopulmonary connection. We sought to quantify the unloading of the single ventricle of left ventricular. (LV) morphology by 3DE after superior cavopulmonary anastomosis. (SCPA) or Fontan operation over a period of 3 months and thereby derive a preliminary 3DE data set on this patient subset. PATIENTS AND METHODS: Eighteen patients with functional single ventricle of LV morphology, who underwent SCPA or completion of Fontan circulation, were included in the study. Volume of the ventricle was estimated by 3DE before surgery and after surgery. (in the early postoperative phase and 3 months after surgery), and indexed end-diastolic volume. (EDV), end-systolic volume. (ESV), and ejection fraction. (EF) were derived. RESULTS: Twelve patients underwent SCPA and six patients underwent staged completion of Fontan circulation. Before surgery, EDV was similar in both groups. There was a significant fall in EDV immediately after SCPA (from 48.3 ± 14.9 ml/m2 to 39.5 ± 12.3 ml/m2). However, EDV increased at 3 months' follow-up to 41.3 ± 10.5 ml/m2. There was no significant fall in EDV immediately after Fontan operation (47.2 ± 10.1 ml/m2-46.6 ± 14.2 ml/m2), but EDV continued to fall at 3 months of follow-up (44.7 ± 10. ml/m2). There was no significant change in ESV in either group, but EF fell significantly after SCPA. CONCLUSIONS: We provide preliminary information on 3DE volume data of single ventricle of LV morphology and the pattern of unloading after SCPA and Fontan operation. Immediate significant volume unloading occurred after SCPA which tended to catch-up after 3 months, whereas continued fall in ventricular volume with time was noted after Fontan.

Imaging the elusive anomalous origin of left coronary artery from pulmonary artery.

A 7-year-old girl with severe mitral regurgitation and pulmonary artery hypertension with good ventricular function was planned for mitral valve repair, but was subsequently diagnosed to have anomalous left coronary artery from pulmonary artery. We describe the pitfalls in the diagnosis of this rare anomaly, particularly in the presence of severe pulmonary artery hypertension and absence of circumstantial evidence of coronary steal and ventricular dysfunction.

Regression of pulmonary vascular disease after therapy of Abernethy malformation in visceral heterotaxy.

A 1-year-old boy who had left isomerism and corrected transposition of the great arteries (c-TGA) with moderate-sized ventricular septal defect, severe pulmonary artery hypertension (PAH), and pulmonary vascular disease with significant right-to-left shunting received a diagnosis of type 2 Abernethy malformation, which was partly responsible for disproportionate PAH in the child. The malformation was treated by plugging of the portosystemic shunt. Follow-up cardiac catheterization on sildenafil demonstrated significant left-to-right shunting (2.16:1) and a fall in pulmonary vascular resistance, making surgical correction possible. This case highlights the importance of searching for additional rare causes of PAH in patients with congenital heart diseases when the degree of pulmonary hypertension is disproportional to the defect size.