Between January 2002 and December 2012, five patients (4 female) underwent corrective surgery for anomalous left coronary artery from pulmonary artery (ALCAPA). They were older than 1 year (range, 3-56 years). One of the 2 patients younger than 10 years had presented with congestive heart failure, and the other had experienced repeated episodes of lower respiratory tract infection since childhood. Of the remaining 3 adult patients, 2 had experienced angina with effort, and 1 patient had had repeated respiratory tract infections since childhood, with mild dyspnea on effort of New York Heart Association (NYHA) class II. Three patients had the anomalous left coronary artery implanted directly into the ascending aorta via coronary-button transfer, and 2 patients underwent coronary artery bypass with obliteration of the left main ostium. Two patients underwent concomitant mitral valve repair procedures, and 1 patient underwent direct closure of a perimembranous ventricular septal defect. Four patients survived the surgery, and 1 patient died because of a persistently low cardiac output. Follow-up times ranged from 3 months to 4 years. All survivors are in NYHA class I and have left ventricular ejection fractions of 45% to 60%, with moderate (n = 1), mild (n = 1), or no (n = 2) mitral insufficiency. We conclude that a few naturally selected patients with ALCAPA do survive beyond infancy and can undergo establishment of 2 coronary systems with satisfactory results.

Objective: To evaluate the relationship between myocardial histopathology and tissue Doppler imaging (TDI) variables of the right ventricle and postoperative peak systolic right-to-left ventricular pressure ratio (Prv/Plv) in patients undergoing intracardiac repair for tetralogy of Fallot (TOF).Methods: Operatively resected crista supraventricularis muscle specimens from 93 patients undergoing intracardiac repair for TOF, aged 18 months to 26 years (mean, 7.02 AE 5.35 years) were subjected to light microscopy. TDI-derived parameters between the normal and abnormal categories of myocardium, the evolution of Prv/Plv, and its relationship to TDI-derived variables were tested using generalized linear random effects model using xtreg command.Results: The incidence of myocyte hypertrophy, myocytolysis, and perivascular fibrosis was 89.2%, 83.8%, and 77.4%, respectively. Although tricuspid annular peak systolic excursion, peak myocardial velocity during systole (s 0 ), and early diastolic basal lengthening of right ventricle (e 0 ) continued to improve among patients with myocardial hypertrophy, myocytolysis, and perivascular fibrosis, there was an absence of improvement of the late diastolic relaxation of right ventricular free wall (a 0 ) in patients with perivascular fibrosis. Although there was improvement of postoperative Prv/Plv in patients with myocardial fibrosis as compared with normal histology, the values were not statistically significant (b [standard error] À0.07 [0.08], P ¼ .3). Conclusions:The great majority of myocardial tissues in cyanotic TOF indicate pre-existing hypertrophic, degenerative, and fibrotic changes. Perivascular fibrosis affects the diastolic compliance of the right ventricle and may account for the absence of improvement of late diastolic relaxation (a 0 ) and greater postoperative Prv/Plv in the absence of a residual surgical lesion.

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We report an unusual cause of failure of successful delivery of antegrade cardioplegia through the aortic root that was caused by the accidental passage of the left ventricular vent catheter across the aortic valve producing acute aortic regurgitation. This problem is best prevented.