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, computedtomographic 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.

OBJECTIVES The most severe combinations of cardiac malformations exist in individuals having jumbled-up thoracic and abdominal organs. These patients make up 2 distinct syndromes. As yet, the consensus is lacking on how best to describe the subsets. The subsets are frequently grouped together in terms of ‘heterotaxy’. The surgical approaches to the subsets, however, are markedly different. We reviewed our experiences with regard to the anatomy as observed in the autopsy room, by the analysis of computed tomographic studies, and in the operating room, to assess whether the lesions might be segregated on the basis of isomerism of the atrial appendages. METHODS AND RESULTS A review of our findings from the examination of specimens from several archives, along with investigation of a large cohort of patients being prepared for surgical treatment, showed that individuals can uniformly be segregated into subgroups on the basis of isomeric arrangement of the atrial appendages. In all instances, this was made possible by using the criterion of the extent of the pectinate muscles within the appendages as judged relative to the atrial vestibules. Segregation on this basis, which correlated excellently with the bronchial arrangement, sets the scene for an appropriate description of the remainder of the heart, providing the cardiac surgeon with all the inferences required for appropriate surgical intervention. CONCLUSIONS When assessing individuals having the features of so-called ‘heterotaxy’, it is possible to segregate the groups into subsets of individuals having either isomeric right or left atrial appendages. This approach provides the framework for the assessment of appropriate surgical management.

We present a synthesis of 95 published investigations of the exceedingly rare tunnels that can exist between the aortic root and the left or right ventricles. From the 220 suitable cases included in these investigations, we reviewed the clinical presentations, modalities used for diagnosis, surgical approaches, and outcomes. Diagnostic information was provided by clinical presentation, radiographic findings, saline contrast echocardiography, computed tomographic angiocardiography, magnetic resonance imaging, cardiac catheterization, and angiocardiography. These techniques elucidated the coronary arterial origins and associated defects and defined the disease before surgery. Patients occasionally present with an asymptomatic cardiac murmur and cardiomegaly, but most suffer cardiac failure in the first year of life when the tunnel enters the left ventricle. Antenatal diagnosis by fetal echocardiography is reliable after 18 weeks of gestation. Associated defects, involving the proximal coronary arteries or the aortic or pulmonary valves, are present in nearly half the cases. Prompt diagnosis and surgical repair are important for a favorable outcome. Overall, operative mortality has been cited to be between 3% and 8.3%. Associated congenital coronary arterial anomalies, residual severe aortic stenosis, poor left ventricular function, and rupture of an infected suture line have been the reported causes of death. Despite early surgical intervention, an incidence of 16% to 60% postoperative residual aortic regurgitation of varying severity has been reported. The requirement of further repair or replacement of the aortic valve ranges from 0% to 50%. We submit that an increased appreciation of these details relative to the tunnels will contribute to improved surgical management.

Objective(s) To determine the influence of surgical techniques adopted to avoid suture line disruption, periprosthetic leakage, patch dislodgement, pericardial patch aneurysm formation, and the long-term stability of aortic root enlargement (ARE) during aortic valve replacement (AVR). Methods One hundred fifteen patients undergoing AVR or combined aortic and mitral valve replacements with Nicks' posterior ARE between 1997 and 2019 underwent long-term echocardiographic and angio-computed tomographic evaluation. Age was 11-72 years (AVR: median, 30; interquartile range, 21-47 years; AVR and mitral valve replacement: median, 27.5; interquartile range, 20-37.5 years). The aortotomy was closed using autologous pericardial patch and Teflon-buttressed sutures. Results Hospital mortality was 1.7% (n = 2), with 4 (3.5%) late deaths. At a mean follow-up of 123.11 ± 77.67 months, the survival probability from Kaplan–Meier was 93.25 ± 0.03%. No cases of severe prosthesis–patient mismatch (PPM) were observed, and only 2 patients had moderate PPM. Median aortic root diameters at the level of sinus of Valsalva and sinotubular junction were 32 (29-35) mm and 33 (30-36) mm, respectively, at discharge, and were 33 (30-36) mm, and 33 (31-37) mm, respectively, at latest follow-up, with no cases of late pericardial patch aneurysm. Conclusions ARE is a safe adjunct to AVR in patients with a small aortic annulus to prevent PPM. Retention of a pericardial collar and Teflon-buttressed sutures is an expedient, safe, and effective technique in reducing bleeding at the enlarged ventriculo-aortic junction. Autologous pericardial patch aortoplasty is not associated with late aneurysm/pseudoaneurysm formation.

Background and Aim To review the anatomical details, diagnostic challenges, associated cardiovascular anomalies, and techniques and outcomes of management, including re‐interventions, for the rare instances of transposition physiology with concordant ventriculo‐arterial connections. Methods We reviewed clinical and necropsy studies on diagnosis and surgical treatment of individuals with transposition physiology and concordant ventriculo‐arterial connections, analyzing also individuals with comparable flow patterns in the setting of isomerism. Results Among reported cases, just over two‐thirds were diagnosed during surgery, after initial palliation, or after necropsy. Of the patients, four‐fifths presented in infancy with either cyanosis or congestive cardiac failure, with complex associated cardiac malformations. Nearly half had ventricular septal defects, and one‐fifth had abnormalities of the tricuspid valve, including hypoplasia of the morphologically right ventricle. A small minority had common atrioventricular junctions We included cases reported with isomerism when the flow patterns were comparable, although the atrioventricular connections are mixed in this setting. Management mostly involved construction of intraatrial baffles, along with correction of coexisting anomalies, either together or multistaged. Overall mortality was 25%, with one‐fifth of patients requiring pacemakers for surgically‐induced heart block. The majority of survivors were in good functional state. Conclusions The flow patterns produced by discordant atrioventricular and concordant ventriculo‐arterial connections remain an important, albeit rare, indication for atrial redirection or hemi‐Mustard's procedure with bidirectional Glenn. The procedure recruits the morphologically left ventricle in the systemic circuit, producing good long‐term functional results. The approach can also be used for those with isomeric atrial appendages and comparable hemodynamic circuits.