Background and Aim: On the basis of previously published accounts, coupled with our own experience, we have assessed the surgical approaches to patients with isomeric atrial appendages.
Methods:We reviewed pertinent published studies on surgical treatment of individuals with isomeric atrial appendages, with the pertinent surgical details provided by most of the manuscripts.Results: Half of patients with right isomerism, and two-thirds of those with left isomerism have bilateral superior caval veins. Azygos extension of the inferior caval vein is reported in three-quarters of those with left isomerism. The coronary sinus is universally absent in right isomerism, along with totally anomalous pulmonary venous connection, and is absent in two-fifths of those with left isomerism. Univentricular atrioventricular connections are expected in up to three-quarters of those with right isomerism. Atrioventricular septal defect is reported in up to fourfifths, more frequently in right isomerism, with such patients typically having discordant ventriculoatrial connections or double outlet right ventricle. Reported mortalities extend to 85% for those with right, and 50% for those with left isomerism. In right isomerism, mortality is up to 54% for systemic-to-pulmonary arterial shunting, up to 75% for univentricular repair, and up to 95% for repair of totally anomalous pulmonary venous connection itself. No more than one-quarter had undergone Fontan completion, with reported mortalities of 21%.
Conclusion:Early surgical results are satisfactory in patients with left isomerism, but disappointing for those with right. Recent advances in cardiac and liver transplantation may offer improved survival.
The issue of operability in patients with shunt lesions and raised pulmonary vascular resistance is contentious. Several reports suggest that patients traditionally considered inoperable may be operated after treatment with targeted drug therapy for pulmonary arterial hypertension. We reviewed all the published literature of “treat and repair” approach to gain more insights into the utility of this approach. A critical appraisal of the published literature suggests that this approach is less established for patients with post tricuspid shunts, and for patients with pre-tricuspid shunts with modestly elevated indexed pulmonary vascular resistance (possibly greater than 11 WU.m2). Targeted drug therapy may be able to extend the therapeutic window in carefully selected patients, but its use as a routine in this setting seems unwarranted.
Background and Aim
We showed in our anatomical review, ventricular septal defects existing as multiple entities can be considered in terms of three major subsets. We address here the diagnostic challenges, associated anomalies, the role and techniques of surgical instead of interventional closure, and the outcomes, including reinterventions, for each subset.
Methods
We reviewed 80 published investigations, noting radiographic findings, and the results of clinical imaging elucidating the location, number, size of septal defects, associated anomalies, and the effect of severe pulmonary hypertension.
Results
Overall, perioperative mortality for treatment of residual multiple defects has been cited to be between 0% and 14.2%, with morbidity estimated between 6% and 13%. Perioperative mortality is twice as high for perimembranous compared to muscular defects, with the need for reoperation being over four times higher. Perventricular hybrid approaches are useful for the closure of high anterior or apical defects. Overall, the results have been unsatisfactory. Pooled data reveals incidences between 2.8% and 45% for device‐related adverse events. Currently, however, outcomes cannot be assessed on the basis of the different anatomical subsets.
Conclusions
We have addressed the approaches, and the results, of therapeutic treatment in terms of coexisting discrete defects, the Swiss‐cheese septum, and the arrangement in which a solitary apical muscular defect gives the impression of multiple defects when viewed from the right ventricular aspect. Treatment should vary according to the specific combination of defects.
We report a case of a 3‐month‐old boy transposition of great arteries, ventricular septal defect, and pulmonary stenosis where computed tomography (CT) angiography detected a “levoatriocardinal vein” connecting the left superior pulmonary vein and the left brachiocephalic vein with a preserved normal connection between the left superior pulmonary vein and left atrium. This case highlights the hemodynamic implications of a “levoatriocardinal vein” as well as the role of CT angiography in detecting such associated cardiovascular anomalies in patients with complex congenital heart defects.
Acute viral infection in children with CF affected course of illness on follow-up, including frequent and severe pulmonary exacerbations requiring hospitalization, intravenous antibiotics, decline in CF scores and increased mortality over next 12-18 months.
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