Aneurysms of Aortic Sinus of Valsalva Dissecting through the Interventricular Septum With Rupture into the Left Ventricle: Case Series and Literature Review.

Aneurysm of Aortic sinus of Valsalva (ASOV) dissecting into the interventricular septum (IVS) and rupturing into the left ventricle (LV) is a rare clinical diagnosis. Systemic inflammatory diseases like tuberculosis can aggravate this condition. We describe three cases of ASOV dissecting into the IVS and rupturing into the LV. All three patients underwent surgical intervention; two had a successful outcome. A literature review was conducted and19 previously reported cases were studied. The extent and direction of septal dissection determined the associated cardiac valvular and rhythm problems. Patch closure of the mouth of the aneurysm is the surgical method of choice. In the presence of multiple sinus tracts or if there is recurrence after surgical closure, aortic sinus or root replacement techniques have better outcomes.

Aortic Origin of a Branch Pulmonary Artery: Early and Mid-Term Single Center Outcomes.

BACKGROUND: Anomalous origin of pulmonary artery (AOPA) is a rare congenital cardiac anomaly. It requires early surgical intervention (<6 months) to prevent irreversible pulmonary vaso-occlusive disease. This study was conducted to determine the surgical outcomes of this rare and intriguing anomaly. METHODS: From January 2015 to 2022, we have studied, 20 patients who underwent surgical correction for this anomaly with a mean age of 6.25 ± 3.7 months. There were 12 patients of <6 months of age. Their preoperative, intraoperative, and postoperative data were collected. They were followed up at every three months for the first year, every six months for two years, and then annually. RESULTS: Among three early mortalities, two were operated before six months of age and one was older than six months (ten months). Those patients presented after six months had longer ventilation time (P = .001). There was no difference in their pulmonary artery pressure (P = .06), right ventricle systolic pressure (RVSP) (P = .85), postoperative saturation (P = .51), inotropic score (P = .06), hospital and intensive care unit stay (P > .05), or mortality (P = .79). There was no late mortality at mean follow-up of 51.31 ± 20.27 months with Kaplan-Meier survival of 85% at 1, 5, and ten years. All patients were asymptomatic, with normal biventricular function and RVSP. One patient required balloon dilatation of the anastomotic site, with Kaplan-Meier event-free survival of 100% at one year, 92% at five and ten years. CONCLUSION: Surgical correction of AOPA in patients beyond six months is still feasible with a higher early morbidity and comparable mortality with good clinical and echocardiographical outcomes at mid-term follow-up.

Iatrogenic dissection in paediatric cardiac surgery.

Iatrogenic dissection due to aortic cannulation is extremely rare. We are reporting here a case of 11-month-old child who developed an iatrogenic aortic dissection during repair of supracardiac total anomalous pulmonary venous connection (TAPVC).

Acute pulmonary artery thromboembolism in presence of large mobile right atrial thrombus and severe thrombocytopenia.

Acute pulmonary embolism in presence of thrombocytopenia poses a challenging situation to manage. Concomitant presence of right atrial thrombus and thrombocytopenia will further complicate the situation. We hereby report a case of large right atrial thrombus with massive saddle bilateral pulmonary artery embolism with severe thrombocytopenia managed surgically with successful outcome.

Measures to improve in-hospital outcomes of patients undergoing surgical repair for anomalous origin of left coronary artery from pulmonary artery.

Background: Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital anomaly leading to progressive left ventricular dysfunction and mitral regurgitation. We conducted this study to investigate various measures to optimize the outcomes of surgical correction for ALCAPA. Materials And Methods: This was a single-centre, retrospective, observational study including consecutive patients operated for ALCAPA. The main outcomes evaluated were in-hospital mortality, duration of mechanical ventilation, and duration of intensive care unit (ICU) stay. Independent sample t- test and Fisher's exact test were used for the analysis of continuous and categorical variables respectively. Results: 31 patients underwent surgical correction for ALCAPA during the study duration. The median age was 7.3 months with a range of 21 days to 25 months. All patients underwent coronary re-implantation with the coronary button transfer technique. There was no in-hospital mortality, the mean duration of mechanical ventilation and ICU stay was 117.6 hours and 10.7 days respectively. Age at admission, development of acute kidney injury after surgery, lactate levels at 12- and 24-hours post-surgery, and heart rate at ICU admission and 12-hours post-surgery were significantly associated with mechanical ventilation duration longer than 48 hours. Use of a combination of levosimendan and milrinone and elective intermittent nasal continuous positive airway pressure ventilation after extubation in all patients with severe left ventricular dysfunction were helpful in preventing low cardiac output and need for reintubation post-surgery respectively. Conclusion: Surgical correction for ALCAPA by coronary re-implantation has an excellent short-term outcome. Optimal postoperative management is of utmost importance for achieving the best results.

Subpulmonary pseudoaneurysm-a rare complication following surgical ventricular septal defect closure.

In this report, we describe the surgical management of a subpulmonary pseudoaneurysm following surgical closure of ventricular septal defect in an infant. Diagnosis, pathogenesis, and surgical considerations for management of this complication are discussed.

Concomitant pulmonary valve replacement with intracardiac repair for adult tetralogy of Fallot.

OBJECTIVES: Adult patients undergoing tetralogy of Fallot (TOF) repair have a higher risk of mortality compared to pediatric patients. Pulmonary regurgitation (PR) further predisposes these patients to heart failure, arrhythmias, and sudden death. Pulmonary valve replacement (PVR) may improve the symptoms in these patients but, fails to reverse the other deleterious effects. Aim of our study was to evaluate the effect of concomitant PVR with TOF repair on right ventricular (RV) parameters, cardiopulmonary exercise capacity, and bioprosthetic valve durability at mid-term. MATERIALS AND METHODS: Between January 2013 and August 2018, 37 adolescents and adults with TOF who had hypoplastic pulmonary annulus underwent concomitant TOF repair with PVR at our institute. We retrospectively collected the data from the hospital records including follow-up. RESULTS: Mean age of the patients was 18.48 ± 7.53 years. Bioprosthetic valve size ranged from 19 mm to 25 mm. There was no early or late mortality. No patient had developed significant perioperative complications. At a mean follow-up of 53.3 ± 16.4 months, there was no significant change in mean QRS duration, RV function, RV end-systolic and end-diastolic dimensions, RV myocardial performance index, and functional status (including NYHA class and 6-min walk test) compared to at-discharge values. Four patients developed prosthetic valve degeneration with mild PR and without significant increase in gradient. CONCLUSION: Concomitant PVR with TOF repair in adult provides excellent mid-term outcome, with a minimal rate of pulmonary valve degeneration. It not only eases the early postoperative course but also preserves the RV function as well as functional status at mid-term.

Venoarterial extra corporeal membrane oxygenation and blood component usage in pediatric patients undergoing cardiac surgery: Single centre experience.

Background: Extra Corporeal Membrane Oxygenation (ECMO) is a well-known tool for providing life-saving support in patients developing post cardiotomy cardiogenic shock in post cardiac surgeries. The current study was designed to evaluate blood transfusion requirements and its relation to mortality in neonate and pediatric cardiac patients requiring venoarterial cardiac ECMO during post-operative period following cardiac surgery. Materials and Methods: Overall 24 pediatric patients (including neonates) who underwent VA ECMO in post cardiac surgery at our institute from January 2016 to October 2017 were included in the study. The details of demographics, blood transfusion, ECMO, and morbidity and mortality were collected for all the patients. Objective of the Study: The primary objective of our study was to assess the outcome of patients on ECMO in post pediatric cardiac surgery. The secondary objective of the study was to assess the effect of blood transfusion on the outcome of the patients. Results: Overall mortality rate was 50% (n = 12). The overall transfusion rate of packed red blood cells was higher in patients who did not survive even after institution of VA ECMO. The transfusion of other blood products like platelets, cryoprecipitate, and fresh frozen plasma were also higher in this group of patients though it was statistically non-significant except for packed red cell transfusion. Though statistically non-significant, the patients who didn't survive even after institution of VA ECMO post-surgery had relatively higher mean age (703.88 ± 998.94 days) as compared to their counterparts (510.63 ± 384.36 days). Conclusion: The use of ECMO is associated with considerable morbidity and mortality. Packed red cell transfusion is definitely higher in expired patients, indicative of deteriorated status of the patient. However, considering non-significant association of other blood components, except packed red cell it is recommended that patients' overall clinical condition should be taken into consideration for transfusion of blood products and not only targeting the transfusion triggers.

Levoatriocardinal Vein With Multiple Ventricular Septal Defects and Without Left-Sided Valvular Atresia.

Levoatriocardinal vein without left-sided valvular atresia is rare. We hereby present an image of the levoatriocardinal vein in a patient with multiple muscular ventricular septal defect with small atrial septal defect and mitral regurgitation.

Mid-Term Outcome of Right Ventricle to Pulmonary Artery Shunt for Older Children and Young Adults With Ventricular Septal Defect, Pulmonary Atresia, and Hypoplastic Pulmonary Arteries.

Management strategy for patients of ventricular septal defect and pulmonary atresia (VSD/PA) with hypoplastic pulmonary arteries presenting in late childhood or adolescence is still controversial. We present our experience with the use of right ventricle-pulmonary artery shunt (RV-PA) in management of this entity. Between January 2014 and April 2018, 25 patients of VSD/PA underwent valveless RV-PA shunt at our center. The size of the RV to PA shunt was calculated as half the expected diameter of the main pulmonary artery. We retrospectively reviewed the data from hospital records. Follow-up data were recorded from outpatient records or via telephone. Mean age of the cohort was 12.25 ± 3.18 years. There was 1 early and 1 interstage mortality. None of the patient developed acute renal failure, ventricular dysfunction, and arrhythmias. At interstage follow-up of 8.28 ± 3.7 months, both Nakata index (from 66.23 ± 24.12 to 185.8 ± 58 mm2/m2) and McGoon ratio (0.9 ± 0.22 vs 49 1.84 ± 0.4) increased significantly compared to preoperative value, whereas RPA-LPA ratio was not significantly changed (1.095 ± 0.39 vs 1.01 ± 0.56, P = 0.63). Prerepair pulmonary vascular resistance in 17 patients, who underwent complete repair, was 2.9 ± 0.69 woods unit/m2. Postrepair right ventricle-left ventricle pressure ratio was 0.5 ± 0.14. There was no early or late mortality and none of the patient required conduit revision or VSD fenestration. On follow-up of 25.75 ± 17.94 months, 16 patients were in NYHA I and 1 patient was in NYHA II. Appropriate-sized RV-PA shunt is an effective strategy for achieving balanced pulmonary artery growth in VSD/PA with hypoplastic pulmonary arteries presenting late without the risk of pulmonary over circulation or systemic malperfusion.

Surgical management of aortopulmonary window: 24 years of experience and lessons learned.

OBJECTIVES: Aortopulmonary window represents 0.2-0.3% of all congenital heart lesions. Progressive pulmonary arterial hypertension and its consequences are more common with this anomaly. The purpose of this study was to share 24 years of surgical experience in managing a spectrum of 55 cases of aortopulmonary window, followed up to 17 years in a single institution. METHODS: This retrospective study was done from November 1991 to November 2015 of 55 patients with aortopulmonary window who underwent successful surgical repair. Age ranged from 5 months to 31 years with 45 children (12 years and younger) and 10 adults (older than 12 years). The male:female ratio was 2.2:1. The mean weight at operation was 14.63 kg (range 3.5-50 kg). An initial diagnosis was obtained from 2D echocardiography, which showed echo dropout in the parasternal short-axis view. Cardiac catheterization and angiography were performed in 54 out of 55 patients. Cardiac catheterization was not done in 1 patient who was 4 months of age. The mean right ventricular systolic pressure (RVSP) was 94 ± 2 mmHg, and the pulmonary artery mean pressure was 68 ± 2 mmHg. The average left to right shunt was 5.2:1, and the pulmonary vascular resistance index in room air was 7.97 ± 0.5 Wood units, whereas after oxygen administration, it declined to 2.0 ± 0.5 Wood units. Four surgical techniques were used based on the size of the communication and the anatomical conditions. RESULTS: There were no early or late deaths. There were no pulmonary hypertensive crises. All patients underwent echocardiography before discharge; none showed a residual shunt. Mild left ventricular dysfunction was seen in 2 patients. This dysfunction regressed with afterload reduction and diuretics on follow-up. All patients were followed up at intervals of 3 months, 1, 5 and 10 years, with the longest follow-up being 17 years. The mean follow-up period was 7 years. At follow-up, all patients were New York Heart Association class I. The mean RVSP on echocardiography was 32 mmHg at 3 months and 30 mmHg at 7 years with no change on further follow-up. Residual pulmonary hypertension was seen in 3 patients: 2 had mild pulmonary hypertension at 8-years follow-up and 1 had moderate hypertension at 3-months follow-up who required sildenafil postoperatively. CONCLUSIONS: Aortopulmonary window is a rare but well identified and surgically correctable anomaly. Operative repair should be offered as soon as the diagnosis is established, regardless of the patient's age. Irreversible pulmonary hypertension with a right to left shunt despite oxygen administration is the only contraindication for surgery. Various surgical techniques can be applied depending on the size of the communication. Associated arch anomalies may require technically challenging approaches and surgical strategies. Early and long-term outcomes after surgical correction are excellent regardless of age or pulmonary vascular resistance.

Crisscross heart-Morphology, clinical diagnosis, and management options.

Crisscross heart is a rare cardiac malformation characterized by crossing of the inflow streams of the two ventricles due to a twisting of the heart about its long axis. The developmental mechanisms and causes of crisscross heart remain unknown. Neonates present with cyanosis and a systolic murmur. In this study, we report a case of crisscross heart with situs inversus, concordant atrioventricular and ventriculo arterial connection with ventricular septal defect and pulmonary stenosis, and review the literature on this rare cardiac malformation.

Accessory Tricuspid Valve Leaflet-Morphology, Diagnosis, and Management.

Accessory tricuspid valve tissue is a rare entity associated with congenital cardiac anomalies. We report a seven-month-old child who underwent ventricular septal defect closure with excision of an accessory tricuspid valve leaflet and discuss their morphology, clinical presentation, diagnostic modalities, and treatment options. doi: 10.1111/jocs.12731 (J Card Surg 2016;31:324-326).

Aorto-left atrial tunnel: a rare entity.

Aorto-left atrial tunnel (ALAT) is a vascular channel that originates from 1 of the sinuses of Valsalva and terminates in the left atrium. The aorto-left atrial tunnel is an extremely rare anomaly. We describe here a case of congenital aorto-left atrial tunnel in a 4-year-old child who underwent successful surgical ligation with good immediate and early results.

Total anomalous systemic with partial anomalous pulmonary venous connections.

A 9-year-old girl with cyanosis, dyspnea, and grade II clubbing was diagnosed by contrast transthoracic echocardiography and angiocardiography to have an anomalous connection of the venae cavae to the physiologic left atrium with partial anomalous pulmonary venous connection. Successful surgical correction was achieved, and the patient's recovery was uneventful.

Surgical management of supravalvar aortic stenosis with coronary artery involvement.

Supravalvar aortic stenosis is characterized by an obstruction of the left ventricular outflow tract distal to the aortic valve. Its association with left main coronary artery stenosis is well known but right coronary artery involvement has not been reported. We describe two cases of supravalvar aortic stenosis with coronary artery stenosis and its surgical management.

Repair of a distal ductal origin of the left pulmonary artery associated with a ventricular septal defect.

Absence of the main branch of the pulmonary artery (PA) with an intracardiac defect is commonly associated with conotruncal cardiac anomaly. It is rarely associated with a ventricular septal defect and PA hypertension. We describe a surgical technique to implant an aberrant left PA to the main PA in a case of a ventricular septal defect, severe pulmonary hypertension, and distal ductal origin of the left PA.