Ruptured sinus of Valsalva aneurysm in a case of Ebstein anomaly: a clinical masquerade of two perfectly balanced lesions.

A combination of a ruptured sinus of Valsalva (RSOV) with Ebstein anomaly is a rare and clinically challenging entity. We describe a case of a 29-year-old female who presented with progressive dyspnoea and epigastric discomfort with symptoms and clinical findings not consistent with any single definitive diagnosis. In the primary echocardiography assessment, she was diagnosed with Ebstein anomaly. On the grounds of several unexplained clinical features and routine review echocardiography by the cardiology team, she was found to have a rare combination of Ebstein anomaly associated with RSOV. A cath study was performed to study the interplay of the hemodynamics of the two lesions, not described before in the literature, and also explain the clinical features not consistent with any one entity. We emphasize the significance of meticulous history-taking and sound clinical evaluation followed by corroboration with the diagnostic modalities for precise diagnosis and timely planned intervention in such rare associations. Supplementary Information: The online version contains supplementary material available at 10.1007/s12055-023-01622-4.

Surgical management of the scimitar syndrome.

BACKGROUND AND AIM: We sought to address the varied anatomical details, the diagnostic challenges, associated cardiopulmonary anomalies, the techniques, and outcomes of management, including re-interventions of scimitar syndrome. METHODS: A total of 92 published investigations of scimitar syndrome were reviewed. Diagnostic information was provided by clinical presentations, radiographic findings, transthoracic and transesophageal echocardiography, computed-tomographic angiography, magnetic resonance imaging, angiocardiography, and ventilation/perfusion scans. These investigations served to elucidate the origin, course, and termination of the scimitar vein, the intracardiac anatomy, the presence of associated defects, and the patterns of any accompanying pulmonary lesions prior to surgical intervention. RESULTS: Of the patients described, up to four-fifths presented during infancy, with cardiac failure, increased pulmonary flow, and pulmonary hypertension. Associated cardiac and extracardiac defects, particularly hypoplasia of the right lung, are present in up to three-quarters of cases. Overall operative mortality has been cited between 4.8% and 5.9%. Mortality was highest in patients with preoperative pulmonary hypertension, and those undergoing surgery in infancy. Despite timely surgical intervention, post-repair obstruction of the scimitar vein, intra-atrial baffle obstruction, or stenosis of the inferior caval vein were reported in up to two-thirds of cases. The venous obstruction could not be related to any particular surgical technique. On long term follow-up, one sixth of patients reported persistent dyspnoea and recurrent respiratory infections. CONCLUSIONS: Any infants presenting with heart failure, right-sided heart, and hypoplastic right lung should be evaluated to exclude the syndrome. An increased appreciation of variables will contribute to improved surgical management.

A rare case of Kawasaki disease with giant coronary artery aneurysm.

Kawasaki disease (KD) is an acute, self-limiting vasculitis that occurs in children of all ages. This was first described by Kawasaki in 1967. Spontaneous regression is observed; however, 25% of patients develop coronary artery aneurysm (CAA). These may result in ischaemic heart disease causing myocardial infarction, rupture leading to pericardial tamponade and distal embolization which culminate in sudden cardiac death. Diagnosis of KD relies on clinical suspicion with no gold standard diagnostic test. A case of KD with giant CAA in a 14-year-old female is described with emphasis on challenges pre- and peri-operatively. The review provided post description of the case emphasizes on pathophysiology with clinical course of CAA in association with KD and justification of our approach with an insight into newer treatment modalities.

Surgical correction of rare anomaly of scimitar syndrome with tetralogy of Fallot.

We present a 9-year-old male with a history of cyanosis diagnosed as scimitar syndrome with tetralogy of Fallot with left anterior descending coronary artery crossing right ventricle outflow tract. He underwent reimplantation of scimitar vein into right atrium baffled into left atrium along with intracardiac repair for tetralogy of Fallot. Postoperative recovery was uneventful and the patient was discharged on postoperative Day 8 and was asymptomatic at follow-up at 6 months.

Pulmonary root translocation in corrected transposition of great arteries with ventricular septal defect and pulmonary stenosis with dextrocardia.

We present 13 years old with history of cyanosis and diagnosis of congenitally corrected transposition of great arteries with ventricle septal defect and pulmonary stenosis with dextrocardia. He underwent modified Senning procedure with pulmonary root translocation. Postoperative recovery was uneventful and was extubated on postoperative day 1. Patient remained in sinus rhythm. Postoperative echocardiography showed good biventricular function with no baffle obstruction and mild pulmonary regurgitation with no significant left and right outflow tract obstruction. Patient was discharged on postoperative day 6 and remained asymptomatic on follow up at 3 months.

Combined Rastelli procedure with supra-coronary ascending aorta replacement.

We present a 5-year-old child with pulmonary atresia palliated at infancy with midline shunt now for complete repair with aneurysmally dilated aorta. Patient underwent a combined Rastelli procedure with supra-coronary ascending aorta replacement with hemi-arch repair. Patient was discharged on post-operative day 10 and was asymptomatic on follow-up.

Tetralogy of Fallot with congenital diaphragmatic hernia with right lung aplasia-a rare entity.

A combination of tetralogy of Fallot with diaphragmatic hernia represents a very rare entity. We present a case of 18-month-old child presenting late with cyanosis and respiratory distress. Chest X-ray and computed tomography angiography showed tetralogy of Fallot with small left pulmonary artery with right lung aplasia with congenital diaphragmatic hernia with liver and bowel loops in right chest cavity. The patient was managed with central shunt on cardiopulmonary bypass and was discharged uneventfully.

Arduous redo made simple by "lateral approach"-pulmonary valve replacement via mini left thoracotomy.

We present a follow-up case of total anomalous pulmonary vein repair and pulmonary valvotomy done 21 years back presented with severe pulmonary regurgitation. Magnetic resonance imaging shows the right ventricle end-diastolic volume and end-systolic volume being 185 mL/m2 and 80 mL/m2 , respectively. In addition to it the patient had had severe kyphoscoliosis causing severe pulmonary restriction. The patient underwent mechanical pulmonary valve replacement through a mini left thoracotomy. The patient had an uneventful recovery was discharged on postoperative day 6, and was in class 1 on follow-up at 3 and 6 months of surgery.

Peritoneal Dialysis in Pediatric Postoperative Cardiac Surgical Patients.

BACKGROUND: We determined the prevalence of acute kidney injury requiring peritoneal dialysis (PD), the factors associated with early PD initiation, prolonged PD and mortality among pediatric postoperative cardiac surgical patients. MATERIALS AND METHODS: The hospital records of 23 children, aged 12 years or younger, who had undergone cardiac surgery and required PD subsequently, during a 1-year period were reviewed. Demographic data, intraoperative variables, and postoperative complications were compared between survivors and nonsurvivors of PD, between the short and long duration PD groups, and between the early and late PD initiation groups. RESULTS: Six hundred and eight pediatric patients who underwent open heart surgery were enrolled in this study. 23 (3.78%) of them required PD. When compared with survivors (n = 11), non survivors (n =12) were more likely to have a higher serum procalcitonin (p = 0.01), higher serum potassium on day 2 (p = 0.001), day 3 (p = 0.04), day of termination of PD (p = 0.001) and a lower urine output on day 3 of PD (p = 0.03). Prolonged PD was associated with time of PD initiation (p = 0.01), a higher postoperative serum creatinine on day 3 (p = 0.01) of PD initiation as well on the day of PD termination (p = 0.01) and the final outcome in terms of survival (p = 0.02). Factors significantly associated with an early PD initiation were CPB time (p = 0.04), sepsis (p = 0.02) and shorter PD duration (p = 0.003). CONCLUSION: PD is very useful mode of renal replacement therapy among pediatric postoperative cardiac surgical patients. The intraoperative and postoperative variables have important association with the time of PD initiation, PD duration and patient survival. HOW TO CITE THIS ARTICLE: Sahu MK, Bipin C, Arora Y, Singh SP, Devagouru V, Rajshekar P, et al. Peritoneal Dialysis in Pediatric Postoperative Cardiac Surgical Patients. Indian J Crit Care Med 2019;23(8):371-375.

Total Anomalous Pulmonary Venous Connection Beyond the First Decade of Life.

OBJECTIVE: We report our experience with surgery for total anomalous pulmonary venous connection (TAPVC) beyond first decade of life. METHODS: Between January 1987 and July 2017, 98 patients ≥ten years underwent TAPVC repair. Their detailed case-records were analyzed. RESULTS: Mean age was 19.05 (± 12.8; median 18) years. Anatomic subtypes were supracardiac (n = 62), cardiac (n = 20), and mixed (n = 16). An atrial septal defect (ASD) was present in all. Severe tricuspid valve regurgitation was present in four patients and severe rheumatic mitral regurgitation was present in one. On preoperative cardiac catheterization, mean pulmonary artery pressure was 67 ± 15.6 mm Hg (median 58; range 37-96). Mean pulmonary vascular resistance was 5.6 ± 3.9 Woods units·m2 (median 4.7, range 2.9-11.8). Twenty-five patients had moderate and eight had severe pulmonary arterial hypertension. Right ventricular dysfunction was present in eight patients. At repair, a small ASD was left open in 87 patients while in 11 patients, the ASD was closed using a unidirectional valved patch. Follow-up was available for 90 (92%) patients. There were no late deaths over a mean follow-up of 163 ± 103.98 months (median 163, range 1-362). Eighty-two patients were in NYHA class I and eight were in class II. Right ventricular function normalized in 82 patients while 80 patients had reduction in pulmonary artery pressure. Event-free survival was 94.2% at 5 years, 92.3% at 10 years, and 90% at 20 and 30 years. CONCLUSIONS: Outcomes of surgical repair for TAPVC beyond first decade of life are satisfactory. Close follow-up, however, is necessary for possible persistence of pulmonary arterial hypertension.

Impact of hibernating and viable myocardium on improvement in perfusion and left ventricular ejection fraction after coronary artery bypass graft.

OBJECTIVES: The association between the extent and degree of perfusion-metabolism mismatch and improvement in perfusion and left ventricular ejection fraction (LVEF) after revascularization was assessed. The secondary aim was to identify the best precoronary artery bypass graft surgery (pre-CABG) PET parameter, if any, to predict the improvement in the perfusion and LVEF after CABG. METHODS AND RESULTS: Overal, 31 patients (mean age: 58+8.3 years) with ischemic left ventricle dysfunction underwent NH3 and F-FDG PET for the assessment of myocardial viability. CABG was performed in these patients and after a mean interval of 3 months, NH3 PET was repeated. The percentages of viable myocardium (VM), hibernating myocardium, degree of mismatch, and LVEF in pre-CABG PET were calculated. These were compared, the median [INCREMENT]LVEF and percent increase in perfusion being 5 (interquartile range: 3-9) and 78.7 (interquartile range: 51.3-100), respectively. No significant association was observed between the severity or extent of perfusion defect/mismatch and improvement in perfusion or LVEF after CABG. Patients with at least 65% VM predicted a 5-unit increase in LVEF at 88.9% sensitivity (P=0.1). CONCLUSION: There was no significant relation between the severity and extent of perfusion-metabolism mismatch with improvement in perfusion and LVEF after CABG. After CABG for ischemic left ventricle dysfunction, VM shows a tendency toward better improvement in LVEF.

An unusual combination of discreet subaortic membrane, aortopulmonary window, severe aortic insufficiency and rheumatic mitral regurgitation.

We report a 15-year-old female patient with an unusual combination of discreet subaortic membrane, aortopulmonary window, severe aortic insufficiency and rheumatic mitral regurgitation.

An alternative technique of atrial septectomy during bidirectional superior cavopulmonary anastomosis.

An atrial septectomy is often required to create or enlarge a pre-existing restrictive atrial septal defect in patients with univentricular hearts undergoing the bidirectional superior cavopulmonary anastomosis. We describe an alternative surgical technique through the transected cardiac end of the superior vena cava without a right atriotomy successfully performed in 26 patients.