Ujjwal Kumar Chowdhury

Background: Multiple techniques have been described to surgically address the presence of an anomalous coronary artery crossing the subpulmonary infundibulum in patients with tetralogy of Fallot, with perioperative mortality and ventricular dysfunction both being higher than for those patients without this complication. Aims: Based on our analysis of previous publications, we used our own experience to illustrate the diagnostic accuracy of computed-tomographic angiography in demonstrating the pertinent anatomical features of the complication. Based on our own surgical experience, we then assessed the efficiency and outcomes of the different surgical strategies designed to mitigate the threat posed by the anomalous coronary arteries. Results: Reported perioperative mortality for these patients continues to remain between 3% and 15%, being 3% at our center. Deaths, when occurring, have been the result of inadvertent injury to the coronary arteries, residual right ventricular outflow tract obstruction, and conduit-related problems. There are varying reports of reoperation following corrective surgery for these patients. Conclusions: Computed-tomographic interrogation is now fully capable of delineating all the pertinent anatomic details of anomalous coronary arteries crossing the subpulmonary infundibulum. With regard to treatment, we suggest that staged palliation will remain a requirement in select instances. Our experience confirms that the trans-tricuspid and trans-pulmonary surgical approach displays the best long-term outcomes by reducing the risks for infundibular obstruction. If feasible, it should be preferred to implantation of a conduit. Translocation of the pulmonary trunk is useful in patients with severely hypoplastic pulmonary ventriculoarterial junctions. In cases where this approach cannot be used, turn back of the pulmonary trunk, or Bonchek's procedure, are additional surgical options.

Surgeons usually close the channels described as “ventricular septal defects.” When both arterial trunks arise from the right ventricle, however, the surgeon will be aware that it is not appropriate to close the channel most frequently described as the “ventricular septal defect.” In this latter setting, furthermore, there is currently no name for the area usually closed during surgery to restore septal integrity. Our previous attempts to emphasize the logical problems created by this situation have not, thus far, been met with uniform approbation. This may reflect the fact that we have not always expressed our concepts using words that are easy to understand. But we continue to believe that words are important if we are to achieve optimal understanding. In this review, therefore, we illustrate those areas that can be closed surgically to restore septal integrity, making a comparison with the defects that provide an outlet for the left ventricle, and hence cannot be closed. To assist understanding, we draw further comparison with the situation in the developing heart, when an area that is initially part of the right ventricle becomes the left ventricular outflow tract subsequent to the completion of septation. We discuss all these features in the setting of the simple perimembranous ventricular septal defect, the defects found in tetralogy of Fallot, and those found in the various forms of double outlet right ventricle. We emphasize the importance to the surgeon of knowing the boundaries around which a patch, or baffle, must be placed to restore septal integrity.

We present here-in a 3-month-old male child with anomalous origin of the right pulmonary artery from the ascending aorta with a left-sided persistent arterial duct who underwent division of the anomalous right pulmonary artery, re-implantation of the same in the pulmonary trunk with concomitant ligation of the arterial duct Post-operative recovery was uneventful.

The phenotypic feature of tetralogy of Fallot is anterocephalad deviation of the muscular outlet septum, or its fibrous remnant, relative to the septoparietal trabeculation, coupled with hypertrophy of septoparietal trabeculations. Although this feature permits recognition of the entity, no two cases are identical. Once diagnosed, treatment is surgical. The results of surgical treatment have improved remarkably over recent decades. The results are now sufficiently excellent, including those in the developing world, that attention is now directed toward avoidance of morbidity, while still seeking, of course to minimize any fatalities due to surgical intervention. It is perhaps surprising that attention thus far has not been directed on the potential significance of phenotypic variation relative to either mortality or morbidity subsequent to surgical correction. The only study we have found specifically addressing this variability focused on the extent of aortic override, and associated malformations, but made no mention of variability in the right ventricular margins of the interventricular communication, nor the substrates for subpulmonary obstruction. In this review, therefore, we assessed the potential significance of known morphological variability to the outcomes of surgical intervention in over 1,000 individuals undergoing correction by the same surgeon in a center of excellence in a developing country. We sought to assess whether the variations were associated with an increased risk of postoperative death, or problems of rhythm. In our hands, double outlet ventriculoarterial connection was associated with increased risk of death, while the presence of a juxta-arterial defect with perimembranous extension was associated with rhythm problems.

We describe herein the surgical technique on a 2.5-years-old male child weighing 10 kilograms diagnosed with a functionally univentricular heart, disproportionately small right subclavian artery, and hypoplastic pulmonary arteries (Mc Goon’s ratio of less than 1.0).5 We interposed a short segment of polytetrafluroethylene graft between the right brachiocephalic artery and the intrapericardial portion of the right pulmonary artery closest to the bifurcation of the pulmonary trunk on the right side.

We present here-in the technical details of harvesting the left radial artery in a 50-year- old man undergoing coronary artery bypass grafting. The radial artery has been harvested atraumatically using a Harmonic scalpel (Ethicon Endosurgery LLC, Johnson & Johnson Company, Guaynaba, Puerto Rico, USA) and two vessel loops by “No touch” technique, thereby preventing perioperative spasm. The postoperative recovery was uneventful.

A 18-months-old male child diagnosed with anomalous origin of left coronary artery from the left posterior pulmonary sinus underwent the modification of the “trapdoor” technique, wherein the anomalous left coronary artery was detached from the pulmonary arterial sinus, and combined aortic and pulmonary arterial flaps were used to augment its length. The postoperative recovery was uneventful.

A 5-week-old male child, weighing 4 kg diagnosed with d-transposition of the great arteries with multiple muscular ventricular septal defects, Yacoub’s type-A coronary arterial pattern, successfully underwent arterial switch operation with Dacron patch closure of ventricular septal defects under moderately hypothermic cardiopulmonary bypass and St. Thomas based cold blood cardioplegia under integrated extracorporeal membrane oxygenation. Postoperatively, he required mechanical circulatory assistance for 72 h. At 12 months of follow-up, there was no mitral or tricuspid regurgitation, no neoaortic valve insufficiency with good biventricular function in Ross clinical score of 2.

Objectives: The indications, timing, and results of the so-called “one-and-a-half ventricle repair”, as a surgical alternative to the creation of the Fontan circulation, or high-risk biventricular repair, currently remain nebulous. We aimed to clarify these issues. Methods: We reviewed a total of 201 investigations, assessing selection of candidates, the need for atrial septal fenestration, the fate of an unligated azygos vein and free pulmonary regurgitation, the concerns regarding reverse pulsatile flow in the superior caval vein, the growth potential and function of the subpulmonary ventricle, and the role of the superior cavopulmonary connections as an interstage procedure prior to biventricular repair, or as a salvage procedure. We also assessed subsequent eligibility for conversion to biventricular repair and long-term functional results. Results: Reported operative mortalities ranged from 3% to 20%, depending on the era of surgical repair with 7% risk of complications due to a pulsatile superior caval vein, up to one-third incidence of supraventricular arrhythmias, and a small risk of anastomotic takedown of the superior cavopulmonary connection. Actuarial survival was between 80% and 90% at 10 years, with two-thirds of patients in good shape after 20 years. We found no reported instances of plastic bronchitis, protein-losing enteropathy, or hepatic cirrhosis. Conclusions: The so-called “one-and-a-half ventricular repair”, which is better described as production of one-and-a-half circulations can be performed as a definitive palliative procedure with an acceptable risk similar to that of conversion to the Fontan circulation. The operation reduces the surgical risk for biventricular repair and reverses the Fontan paradox.

Objectives: To compare the long-term outcomes of biventricular, univentricular, and so-called one-and-one-half ventricular repairs in patients with left and right isomerism. Methods: Surgical correction was undertaken, between 2000 and 2021, in 198 patients with right, and 233 with left isomerism. Results: The median age at operation was 24 days (interquartile range [IQR]: 18-45) and 60 days (IQR: 29-360) for those with right and left isomerism, respectively. Multidetector computed-tomographic angiocardiography demonstrated more than half of those with right isomerism had superior caval venous abnormalities, and one-third had a functionally univentricular heart. Almost four-fifths of those with left isomerism had an interrupted inferior caval vein, and one-third had complete atrioventricular septal defect. Biventricular repair was achieved in two-thirds of those with left isomerism, but under one-quarter with right isomerism ( P < .001). Hazard regression for mortality revealed odds for prematurity at 5.5, pulmonary atresia at 2.81, atrioventricular septal defect with a common valvar orifice at 2.28, parachute mitral valve at 3.73, interrupted inferior caval vein at 0.53, and functionally univentricular heart with a totally anomalous pulmonary venous connection at 3.77. At a median follow-up of 124 months, the probability of survival was 87% for those with left, and 77% for those with right isomerism ( P = .006). Conclusions: Multimodality imaging characterizes and delineates the relevant anatomical details, facilitating surgical management of individuals with isomeric atrial appendages. Continuing higher mortality despite surgical intervention in those with right isomerism points to the need for the reassessment of strategies for management.