How do Age at the Surgery and Birth Weight Influence Post-Operative Anthropometric Parameters in Infants with Surgical Closure of Large Ventricular Septal Defects? A Prospective Cohort Study from a Lower-Middle-Income Country.

Closure of the large ventricular septal defects (VSD) in infancy can lead to normalization of growth, but data are limited. Our study is done to assess the growth pattern in different age groups of children and lower birth weight babies after shunt closure. This is a prospective observational study that included infants with isolated large VSD operated in infancy. Anthropometric data were collected at baseline and at follow-up, and growth patterns were analyzed. 99 infants were included in the study. The mean age and weight at the time of surgery were 6.97 ± 2.79 months and 5.07 ± 1.16 kg, respectively. The mean follow-up duration was 8.99 ± 2.31 months. The weight for age (W/A) was the most adversely affected parameter preoperatively, and there was significant improvement noted in the mean Z score for W/A after shunt closure (- 3.67 ± 1.18 vs. - 1.76 ± 1.14, p = 0.0012). There was improvement in Z-scores for length for age (L/A) and weight for length (W/L), although it was not statistically significant. The infants from all the age groups had statistically significant growth in the anthropometric parameters. The rate of weight gain was maximum in the infants operated below 8 months of age (2-4 months = 3588 g, 5-6 months = 3592 g, 7-8 months = 3606 g, 9-10 months = 2590 g, 11-12 months = 2250 g). Low birth weight and normal birth weight infants had similar Z-scores at the time of surgery and at follow-up in all 3 anthropometric parameters, and birth weight did not affect pre- as well as post-operative growth parameters. Suboptimal improvement in weight and length was seen in 40 and 20% of babies even after successful surgical repair, respectively. Growth failure in infants with a large VSD can be multifactorial. Early surgical closure of the shunt can lead to early normalization of growth parameters and faster catch-up growth. Few babies may fail to demonstrate a positive growth response even after timely surgical correction, and may be related to intrauterine and genetic factors or faulty feeding habits.

Trans-catheter closure of an unusual type of Abernethy malformation in a child presenting with severe pulmonary hypertension.

Abernethy malformation is a congenital extra-hepatic porto-systemic shunt. This malformation is characterized by an abnormal connection between the portal vein or its branches and one of the systemic veins. Though rare, this anomaly can lead to pulmonary hypertension. Drainage of Abernethy malformation into coronary sinus is extremely rare. We describe a child with Abernethy malformation with unusual drainage into coronary sinus. The abnormal channel was successfully closed by trans-catheter technique with normalisation of pulmonary arterial pressures.

Pseudoaneurysm of aorta after transcatheter closure of patent ductus arteriosus.

Aortic pseudoaneurysm is a rare but life-threatening complication after transcatheter closure of patent ductus arteriosus (PDA) with only a few reported cases in literature. We report a case of an 18-month-old child who was diagnosed to have pseudoaneurysm of the aorta after 6 months of transcatheter closure of PDA. It was diagnosed on routine follow-up on echocardiography evaluation. Computed tomography chest is the imaging modality of choice for evaluation of these patients which showed large saccular aneurysm arising from distal aortic arch beyond origin of left subclavian artery. Any aneurysm more than 30 mm should be treated irrespective of symptoms. Treatment modalities are surgical resection of the aneurysm (as in our patient) or thoracic endovascular aortic repair. In our case, surgical resection of aneurysm was performed. Supplementary Information: The online version contains supplementary material available at 10.1007/s12055-023-01619-z.

Clinical profile and outcomes of multisystem inflammatory syndrome in children associated with COVID-19 virus after surgery for congenital heart defects.

Introduction: To study the clinical presentation, laboratory profile, echocardiographic details, management, and outcomes of children who were diagnosed to have multisystem inflammatory syndrome in children (MIS-C) in the immediate postoperative period after surgery for congenital heart defects (CHDs). Materials and Methods: This is a prospective case-control study that included children diagnosed to have MIS-C in the postoperative period based on clinical signs, rise in inflammatory markers, and echocardiographic features of ventricular dysfunction or coronary involvement. Management included intravenous immunoglobulin (IVIG), steroids, and antiplatelet medications in addition to routine postoperative care. Out of the 461 children who underwent surgery for CHD between April 1st, 2021, and November 30th, 2021, 18 children were diagnosed with MIS-C. After the initial routine postoperative course, all 18 children had sudden worsening in clinical and laboratory parameters. Other causes such as bacterial infection were ruled out. All of these children had features of MIS-C with ventricular dilatation and dysfunction, coronary artery involvement, and reactive COVID-19 immunoglobulin G antibody. There was a significant improvement in coronary artery dimensions after IVIG administration (P = 0.001). The involvement of the left main coronary artery was associated with significantly increased length of intensive care unit (ICU) and hospital stay (P = 0.019). Mean ICU and hospital stay was prolonged in the MIS-C group. There were two deaths in this group due to severe left ventricular dysfunction. Conclusions: During the pandemic, a proportion of patients undergoing elective cardiac surgery may develop unexpected worsening in clinical status due to MIS-C. A high index of suspicion and prompt treatment with IVIG and steroids may be helpful in improving outcomes.

Randomized controlled trial to evaluate the effect of prophylactic amiodarone versus dexmedetomidine on reducing the incidence of postoperative junctional ectopic tachycardia after pediatric open heart surgery.

Background: Junctional ectopic tachycardia (JET) is the most common arrhythmia after pediatric open-heart surgeries (OHS), causing high morbidity and mortality. As diagnosis is often missed in patients with minimal hemodynamic instability, its incidence depends on active surveillance. A prospective randomized trial evaluated the efficacy and safety of prophylactic amiodarone and dexmedetomidine to prevent and control postoperative JET. Methods: Consecutive patients aged under 12 years were randomized into amiodarone, dexmedetomidine (initiated during anesthetic induction) and control groups. Outcome measures included incidence of JET, inotropic score, ventilation, and intensive care unit (ICU) duration and hospital stay, as well as adverse drug effects. Results: Two hundred and twenty-five consecutive patients with a median age of 9 months (range 2 days-144 months) and a median weight of 6.3 kg (range 1.8 kg-38 kg) were randomized with 70 patients each to amiodarone and dexmedetomidine groups, and the rest were controls. Ventricular septal defect and Fallot's tetralogy were the common defects. The overall incidence of JET was 16.4%. Syndromic patients, hypokalemia, hypomagnesemia, longer bypass, and cross-clamp duration were the risk factors for JET. Patients with JET had significantly prolonged ventilation (P = 0.043), longer ICU (P = 0.004), and hospital stay (P = 0.034) than those without JET. JET was less frequent in amiodarone (8.5%) and dexmedetomidine (14.2%) groups compared to controls (24.7%) (P = 0.022). Patients receiving amiodarone and dexmedetomidine had significantly lower inotropic requirements, lower ventilation duration (P = 0.008), ICU (P = 0.006), and hospital stay (P = 0.05). Adverse effects such as bradycardia and hypotension after amiodarone and ventricular dysfunction after dexmedetomidine were not significantly different from controls. Conclusion: Prophylactic amiodarone or dexmedetomidine started before OHS is effective and safe for the prevention of postoperative JET.

Use of 3D volume-rendered CT imaging in analysing the anatomy of an unusual type of common arterial trunk.

A common arterial trunk with pulmonary dominance is rare. We report the use of 3D volume-rendered CT imaging to define common arterial trunk with pulmonary arterial dominance and associated abnormalities.

Staged interventional solution for a diagnostic dilemma caused by hypoplastic left ventricle with severe aortic arch hypoplasia.

Duct dependent aortic arch obstruction with borderline left ventricular hypoplasia presents a diagnostic dilemma. If the left ventricle (LV) is adequate to sustain systemic cardiac output without a patent duct, arch obstruction is relieved surgically and duct is divided. Inadequate LVs do not tolerate duct division, and these patients need more complex Norwood type surgeries. However, catheter-based interventions for arch obstruction can retain ductal patency. The progressive changes in anatomy and physiology of a neonate who presented with hypoplastic left heart structures and patent duct with advancing age and modified by serial catheter-based interventions are presented to highlight the use of interventions in this therapeutic dilemma.

Cobra-head and other shape-memory abnormalities of nitinol atrial septal occluders: incidence, predisposing factors, and outcomes.

BACKGROUND: Shape-memory abnormalities are seen in some nitinol atrial septal occluders. Variably described as cobra-head, tulip, and others, their incidence, mechanisms, clinical impact, and outcome have not been systematically analysed. METHODS: We retrospectively reviewed all consecutive device closures in the last 6 years for deformations. Type and size of the occluder, deployment technique, size, and angulation/kinking of the delivery sheath were analysed. Procedural success, duration, and other complications were studied. RESULTS: A total of 112 devices (11.8%) among 950 occluders used in 936 patients showed deformities. Fourteen of 936 received 2 devices. Deformities were transient and self-correcting in 40%. Multivariate analysis showed significant associations with oversized sheaths (p = 0.004), kinked/angulated sheaths (p < 0.001), special deployment techniques (p < 0.001), and twist in the device waist (p = 0.011). Despite more frequent deformities with Figulla (15.6%) and Amplatzer (13.9%) occluders than Cera occluders (6.6%) and larger devices (>24 mm - 14.6%) than smaller devices (less than or equal to 24 mm - 9.7%), they were not significant on multivariate analysis. In vivo manipulations corrected most deformities; nineteen needed in vitro reformations and four needed a change of device. Despite prolongation of the procedure, repeated attempts (mean 2.76 ± 1.7 attempts, with a range from 1 to 9 attempts), and supraventricular tachycardia in two patients, there were no serious adverse effects. CONCLUSIONS: Deformations were frequent in 11.8% of atrial septal occluders on a targeted search. Oversized and angulated/kinked sheaths, special techniques like pulmonary vein deployment and twist in device waist during procedure predisposed to deformities. While most deformities were corrected with manipulations, removal of the device was infrequently needed and change of device was rarely required. Long procedural time and multiple attempts for deployment did not affect procedural success.

In congenitally corrected transposition of great arteries following Fontan surgery, a left ventricle with suprasystemic systolic and high end-diastolic pressures paradoxically preserves right ventricular and tricuspid valve function.

Systemic right ventricular function in congenitally corrected transposition depends on septal geometry. Suprasystemic left ventricular systolic pressures and high end-diastolic pressures after Fontan surgery paradoxically preserve right ventricular function.