Experience with surgical correction of double outlet both ventricles.

Double outlet both ventricles is a rare abnormal ventriculo-arterial malformation in which both great arterial trunks are committed to both the ventricles, albeit now being recognized with increasing frequency. Patients with the lesion present with a spectrum of clinical manifestations. The size and location of the interventricular communication dictate the feasibility of biventricular repair. Literature on the malformation, however, is sparse. We report our experience with five patients, all of whom underwent successful surgical biventricular repair.

Clinical presentation, conventional/4D spatio-temporal image correlation imaging findings, pregnancy and early postnatal outcomes in fetuses having anomalies of systemic venous return in the absence of significant intracardiac defects: A retrospective study from two centres in Southern India.

BACKGROUND: Recent advances in fetal ultrasound imaging, especially four-dimensional (4D) spatio-temporal image correlation techniques permit detailed evaluation of the fetal venous system and its abnormalities. In this report, we present the clinical presentation, conventional/4D spatio-temporal image correlation imaging findings, pregnancy, and early postnatal outcomes in fetuses having anomalies of systemic venous return in the absence of significant intracardiac defects from two centres in southern India. OBJECTIVES: To report the clinical presentation, conventional/4D spatio-temporal image correlation imaging findings, pregnancy, and early postnatal outcomes in fetuses having anomalies of systemic venous return in the absence of significant intracardiac defects from two centres in Southern India. METHODS: Retrospective study (October 2017 to March 2022). All fetuses referred with abnormal cardiac imaging findings who were diagnosed to have anomalies of systemic venous return in the absence of significant intracardiac defects were included. Imaging techniques like 2D with color and 4D spatio-temporal image correlation fetal imaging were used to evaluate systemic venous anomalies. Systemic venous anomalies were grouped into cardinal venous anomalies and umbilico-portosystemic venous anomalies. RESULTS: Thirty-nine fetuses were included; this represented 4.4% of all cardiac anomalies diagnosed during the study period. Cardinal venous anomalies were seen in 29 fetuses (74%); the mean gestation age at diagnosis was 25.5 ± 3.4 weeks. Absent dilated coronary sinus was associated with anomalous drainage of superior caval vein through completely unroofed coronary sinus into the left atrium (n = 3) which resulted in cyanosis after birth in all infants. Among the three cases, one underwent surgery at 6 months of age due to a progressive increase in cyanosis (Spo2 78%) and others are awaiting surgery. A prenatal genetic evaluation was performed in 17 (58.6%). Cardinal venous anomalies were not associated with genetic abnormalities. Live births occurred in all fetuses with cardinal venous anomalies. Umbilico-portosystemic venous anomalies were seen in 10 (26%). The mean gestation age at diagnosis was 26.5 ± 4.5 weeks. Except in a fetus with the extrahepatic portosystemic shunt (umbilical vein to iliac vein), the genetic evaluation was normal in our cohort. Extracardiac anomalies (n = 3/10; 30%) were seen in both intra (n = 1) and extrahepatic portosystemic shunts (n = 2). CONCLUSION: Anomalies of systemic venous return in the absence of significant intracardiac, extracardiac, and genetic abnormalities carried an overall favorable prognosis in our cohort. Precise characterization of anatomic details using advanced imaging techniques helps in the understanding of this complex three-dimensional anatomy and in the prognostication of these anomalies.

Role of intrapulmonary artery septation in univentricular hearts.

The optimal management of functionally univentricular hearts with a significant unbalanced pulmonary blood flow is unclear. The role of cavopulmonary shunt in the rehabilitation of hypoplastic pulmonary arteries is limited. Intrapulmonary artery septation is a technique, wherein a systemic-to-pulmonary artery shunt provides an additional source of blood flow to rehabilitate the hypoplastic pulmonary artery. We report a case of functionally univentricular heart with hypoplastic left pulmonary artery following a cavopulmonary shunt in whom intrapulmonary artery septation resulted in adequate growth of the left pulmonary artery.

The meandering intrapulmonary total anomalous pulmonary venous channel (MITAPVC)-old wine in new bottle or a new variant?

Background: Total anomalous pulmonary venous connection (TAPVC), with an intrapulmonary segment (IPV), a meandering abnormally located confluence and obligatory drainage of one lung into the other before entering the systemic circulation, is a rare anomaly and we term it as the meandering intrapulmonary TAPVC (MITAPVC). Material and methods: We report five patients with an unusual variation of the TAPVC channel. A review of literature was done to identify this association of TAPVC with an intrapulmonary vein and absence of a confluence in its usual location. Results: In our study, 4 patients with neo-confluence creation had excellent outcome while one with partial correction required catheter-based intervention, but succumbed to persistent pulmonary hypertension refractory to therapy. A literature search showed 25 additional such patients. Two groups were noted, one with isolated lesions (N = 16) and the other with heterotaxy or complex intracardiac lesions (N = 14). Of the 20 surgical interventions, only 12 survived, most of them in the isolated group (N = 10). Mortality was due to incomplete surgery (4/4), inappropriate surgery (3/3), and complete and appropriate surgery (1/11) respectively. Conclusion: The MITAPVC is often associated with heterotaxy and complex lesions. However, the isolated version is being increasingly recognised. Non-recognition or inappropriate surgical correction of MITAPVC is associated with fatal outcomes. Evaluation by a computerised tomography (CT) scan, meticulous dissection and demonstration of the entire channel, creation of a neo-confluence and appropriate palliation for the heterotaxy is the key to ensure good outcome. This is not a new entity, but deserves a separate subclassification under TAPVC. Supplementary Information: The online version contains supplementary material available at 10.1007/s12055-021-01290-2.

Total Anomalous Pulmonary Venous Connection Repair: Single-Center Outcomes in a Lower-Middle Income Region.

BACKGROUND: The management of total anomalous pulmonary venous connection (TAPVC) in neonates and infants is resource-intensive. We describe early and follow-up outcomes after surgical repair of isolated TAPVC at a single institution in a resource-limited setting. METHODS: The data of 316 consecutive patients with isolated TAPVC undergoing repair (January 2010-September 2020) were reviewed. The study setting was a tertiary hospital in southern India that provides subsidized or charitable care. Standard surgical technique was used for repair, circulatory arrest was avoided, and suture-less anastomosis was reserved for small or stenotic pulmonary veins. Surgical and postoperative strategies were directed toward minimizing intensive care unit (ICU) stay. RESULTS: 302 (95.6%) patients were infants and 128 patients (40.5%) were neonates; median weight was 3.3 kg (IQR 2.8-4.0 kg). Obstruction of the TAPVC was seen in 176 patients (56%) and pulmonary hypertension in 278 patients (88%). Seventeen (5.4%) underwent delayed sternal closure. The median postoperative ICU stay was 120 h (IQR 96-192 h), mechanical ventilation was 45 h (IQR 24-82 h), and hospital stay was 13 days (IQR 9-17 days). There were three in-hospital deaths (0.9%). Over a median follow-up period of 53.3 months (IQR 22.9-90.4), pulmonary vein restenosis was seen in 32 patients (10.1%) after a mean of 2.2 months (1-6 months). No perioperative risk factors for restenosis were identified. CONCLUSIONS: Using specific perioperative strategies, it is possible to correct TAPVC with excellent surgical outcomes in low-resource environments. Late pulmonary vein restenosis remains an important complication.

Intrapulmonary artery septation for pulmonary artery growth in functionally univentricular hearts.

The pulmonary artery anatomy is one of the important determinants of Fontan completion and its outcomes in patients with functionally univentricular hearts. Despite several modifications to Fontan operation, unbalanced pulmonary arteries pose a significant challenge in this subset of patients. Intrapulmonary artery septation is a technique, developed to rehabilitate the unilateral hypoplastic pulmonary artery following various initial palliative procedures in functionally univentricular hearts. Literature is sparse on this technique. This article reviews the basic concepts and literature on intrapulmonary artery septation. We also describe our experience with modified intrapulmonary artery septation.

Aortic root widening: "pro et contra".

In patients with a small aortic annulus, the clinical benefits of aortic valve replacement depend on avoidance of patient-prosthesis mismatch as it is associated with reduced overall survival. Aortic root widening or enlargement is a useful technique to implant larger valve prosthesis to prevent patient-prosthesis mismatch. Posterior annular enlargement is the commonest technique used for aortic root enlargement. Consistent enlargement of the aortic root requires more extensive procedures like Manouguian or Konno-Rastan techniques. The patients commonly selected are younger patients with good life expectancy. However, caution is advised in applying this procedure in elderly patients, patients with heavily calcified annulus and when performing concomitant procedures. There is no definitive conclusion on the best material to use for the reconstruction of aortic annulus and aorta in aortic root enlargement procedures.

Prenatal diagnosis of transposition of great arteries with left anterior aorta (SDL-TGA).

We report a case with prenatal diagnosis of transposition of great arteries (TGA) with L-posed aorta (SDL-TGA) which was confirmed by postnatal echocardiography. The anatomic findings were confirmed during the successful arterial switch operation (ASO). The technical challenges of ASO in the L-posed aorta are also described.

Circumflex aorta: An uncharted territory.

BACKGROUND: Vascular rings are aortic arch anomalies with a spectrum of manifestations ranging from asymptomatic lesions detected incidentally to an acute presentation secondary to tracheo-esophageal compression. Circumflex retro-esophageal aortic arch is an extreme form of true vascular ring. It remains an uncharted territory to many surgeons. METHODS: A comprehensive search of peer reviewed journals was completed based on the key words, "Circumflex aorta," "Circumflex retro-esophageal aorta" and "circumflex arch" using Google scholar, Scholars Portal Journals and PubMed. The reference section for each article found was searched to obtain additional articles. Literature on the circumflex aorta was reviewed starting from the embryogenesis to the latest management strategies. RESULTS: Right circumflex aorta is more prevalent compared to left circumflex aorta. It can occur in isolation or in association with other intracardiac lesions. Mainly presents in children, however reported in adults too. The presentation may vary from asymptomatic lesion to acute respiratory distress secondary to airway compression. Computerized tomography (CT) and magnetic resonance imaging (MRI) are important tools in delineating the vascular anatomy. Aortic uncrossing is the definitive procedure. However, the role of concomitant tracheobronchopexy is emerging. Native tissue-to-tissue anastomosis is commonly preferred, but cases of extra-anatomic grafts are reported. CONCLUSION: Circumflex aorta is amenable to complete repair. Preoperative delineation of anatomy is important for successful surgical outcome. Division of the retro-esophageal segment is crucial in relieving the compressive symptoms. In addition, tracheobronchopexy is helpful in addressing residual tracheomalacia but this accounts for a high-risk surgery.

Perioperative right ventricular function and dysfunction in adult cardiac surgery-focused review (part 2-management of right ventricular failure).

The single most important factor in improving outcomes in right ventricular (RV) failure is anticipating and recognizing it. Once established, a vicious circle of systemic hypotension, and RV ischemia and dilation, occurs, leading to cardiogenic shock, multi-organ failure, and death. RV dysfunction and failure theoretically can occur in three settings-increase in the pre-load; increase in after load; and decrease in contractility. For patients deemed low risk for the development of RV failure, when it occurs, the correction of underlying cause is the most important and effective treatment strategy. Therapy of RV failure must focus on improving the RV coronary perfusion, lowering pulmonary vascular resistance, and optimizing the pre-load. Pre-load and after-load optimization, ventilator adjustments, and improving the contractility of RV by inotropes are the first line of therapy and should be initiated early to prevent multi-organ damage. Mechanical assist device implantation or circulatory support with extracorporeal membrane oxygenation (ECMO) may be needed in refractory cases.

Perioperative right ventricular function and dysfunction in adult cardiac surgery-focused review (part 1-anatomy, pathophysiology, and diagnosis).

Right ventricle (RV) dysfunction and failure are now increasingly recognized as an important cause of perioperative morbidity and mortality after cardiac surgery. Although RV dysfunction is common, RV failure is very rare (0.1%) after routine cardiac surgery. However, it occurs in 3% of patients after heart transplantation and in up to 30% of patients after left ventricular assist device implantation. Significant RV failure after cardiac surgery has high mortality. Knowledge of RV anatomy and physiology are important for understanding RV dysfunction and failure. Echocardiography and haemodynamic monitoring are the mainstays in the diagnosis of RV dysfunction and failure. While detailed echocardiography assessment of right heart function has been extensively studied and validated in the elective setting, gross estimation of RV chamber size, function, and some easily obtained quantitative parameters on transesophageal echocardiography are useful in the perioperative setting. However, detailed knowledge of echocardiography parameters is still useful in understanding the differences in contractile pattern, ventriculo-arterial coupling, and interventricular dependence that ensue after open cardiac surgery. SUPPLEMENTARY INFORMATION: The online version contains supplementary material available at 10.1007/s12055-021-01240-y.

Aortic uncrossing with concomitant arch augmentation in a rare case of left circumflex aorta with arch hypoplasia.

Retro-esophageal circumflex aortic arch is a rare form of vascular ring. Majority of cases occur in isolation without any intracardiac lesions. Only a few cases of left circumflex aorta have been reported in the literature. We present a case of left circumflex aorta with associated arch hypoplasia and coarctation, ventricular septal defect, who underwent single-stage repair.

Berry syndrome-a rare congenital cardiac anomaly.

Berry syndrome is a rare congenital cardiac anomaly, characterized by distal aortopulmonary window, hypoplasia or interruption of the aortic arch, intact ventricular septum, and aortic origin of the right pulmonary artery and patent ductus arteriosus. Anatomic depiction of each component is important for the diagnosis. Single-stage surgical repair is challenging but feasible with good survival outcomes. The available literature on this anomaly is limited. Hence, this paper aims at reviewing the literature on Berry syndrome.

Prenatal diagnosis of double outlet right ventricle with intact ventricular septum in two foetuses.

We describe two cases of an unusual variant of double outlet right ventricle with intact ventricular septum diagnosed prenatally and confirmed by foetal autopsy in a case. The first case had mitral valve atresia, slit-like left ventricle, and normally related great arteries. The second case had mitral valve atresia, hypoplastic left ventricle, parallel outflows with an interrupted aortic arch.

Transaxillary Approach for Surgical Repair of Simple Congenital Cardiac Lesions: Pitfalls, and Complications.

BACKGROUND: In the treatment of simple congenital cardiac lesions, percutaneous and cosmetic surgical approaches have steadily gained prominence. Surgically, right vertical axillary approach is sparsely used despite superior cosmesis and less pain and blood product usage. Knowledge of potential pitfalls could lead to its more widespread acceptance. METHODS: We retrospectively analyzed perioperative outcomes of 104 consecutive patients who underwent surgery by this technique between mid-2016 and December 2019, including ostium secundum (67), sinus venosus (34), coronary sinus (1), and ostium primum (1) atrial septal defects and hemianomalous pulmonary venous connection (1). Perioperative variables, surgical times, complications, and follow-up data were analyzed. RESULTS: Patient weight ranged from 6.8 to 41 kg. Incision length ranged from 4 to 6 cm. There was no mortality. All cannulation was central. Difficulty in cannulation (inferior vena cava) was seen in two patients. Morbidity included pneumothorax in 2 (1.9%) patients and subcutaneous emphysema necessitating prolonged intercostal drain retention in 20 (19.2%) patients. Surgical time increased linearly (r = 0.567; P < .001) with increasing patient weight but cardiopulmonary bypass (CPB) time remained unaffected. No chest deformities or paresthesia were noted on follow-up. Scar size decreased in some patients. CONCLUSIONS: Right vertical axillary approach can be safely employed to treat simple congenital cardiac lesions with adequate awareness of potential pitfalls. Increasing patient weight increases the surgical time but does not affect CPB times. Incidence of pneumothorax and subcutaneous emphysema is similar to other thoracotomy approaches. It is cosmetically superior.

Prenatal diagnosis of partial absence of the tricuspid valve with perimembranous ventricular septal defect-The utility of prenatal 4-dimensional spatiotemporal image correlation rendering.

First described in 1965 by Johnson and Wilcox, partial absence of the tricuspid valve (TV) associated with a ventricular septal defect (VSD) is a very rare malformation (Ann Surg. 1966;164:334-336). Heart failure secondary to severe TV regurgitation results in utero fetal compromise (J Am Coll Cardiol. 1991;17:167). We present here a fetus with partial absence of the tricuspid valve with perimembranous VSD.

Conversion of prior univentricular repairs to septated circulation: Case selection, challenges, and outcomes.

OBJECTIVES: Complex congenital heart defects that present earlier in life are sometimes channelled in the single ventricle pathway, because of anatomical or logistic challenges involved in biventricular correction. Given the long-term functional and survival advantage, and with the surgeons' improved understanding of the cardiac anatomy, we have consciously explored the feasibility of a biventricular repair in these patients when they presented later for Fontan completion. We present a single institution's 10-year experience in achieving biventricular septation of prior univentricular repairs, the technical and physiological challenges and the surgical outcomes. METHODS: Between June 2010 and December 2019, 246 patients were channelized in the single ventricle pathway, of which 32 patients were identified as potential biventricular candidates at the time of evaluation for Fontan palliation, considering their anatomic feasibility. The surgical technique involves routing of the left ventricle to the aorta across the ventricular septal defect, ensuring an adequate sized right ventricular cavity, establishing right ventricle-pulmonary artery continuity and taking down the Glenn shunt with rerouting of the superior vena cava to the right atrium. This is a retrospective study where we reviewed the unique physiological and surgical characteristics of this subset of patients and analysed their surgical outcomes and complications. RESULTS: Biventricular conversion was achieved in all cases except in 3 patients, who had the Glenn shunt retained leading to a one and a half ventricle repair. The average age of the patients was 4.9 years of whom 18 were male. The average cardiopulmonary bypass time was 371 min with an average cross clamp time of 162 min. There was one mortality in a patient with corrected transposition of great arteries (c-TGA) with extensive arterio-venous malformations (AVMs). At a median follow-up of 60 months, all patients remained symptom free except two with NYHA II symptoms, one being treated for branch pulmonary artery stenosis with balloon dilatation and the other with multiple AVMs who needed coil closure. One patient with branch pulmonary artery (PA) stenosis required balloon dilatation and stent placement. CONCLUSION: The possibility of achieving the surgical goal in this unique subset of patients evolves with the progressive experience of the congenital heart surgeon. Case selection is a crucial aspect in achieving the desired outcome, and this 'borderline' substrate is often recognized at the time of evaluation for the Fontan completion. A comprehensive preoperative imaging and planning helps in achieving the surgical septation and reconnection to achieve the desired physiological circulation. Though technically challenging, the surgery has excellent short- and mid-term outcomes as evidenced by our 10-year experience.