Abstract
BackgroundAnomalous origin of the left coronary artery (LCA) from the pulmonary artery (ALCAPA) is a rare congenital heart disease. Retrograde flow from the right coronary artery (RCA) through natural collaterals to the low-pressure main pulmonary artery causes extensive ischemia. Limited data concerning the extent of permanent myocardial damage and functional recovery after surgical repair in the long-term follow-up is available.AimDetermination of the incidence of incipient myocardial dysfunction in ALCAPA patients in the long-term observation using tissue Doppler and speckle tracking echocardiography.Methods and resultsEighteen ALCAPA patients after surgical repair (at median age of 7 months, range 3–167) underwent echocardiographic examination after (median) 17 years. All but 4 patients in NYHA class II presented well at follow-up. No narrowing in proximal LCA was detected in color Doppler. The initial (pre-surgical) left ventricular (LV) ejection fraction of 33±17% almost normalized to 55±6%, but was lower than in the age, sex and body surface area matched control group: 62±5% (p<0.001). At follow-up, LV global longitudinal strain (LS): -15.8±3.3% vs -21.9±1.7%; right ventricular LS: -20.6±3.9% vs -24.9±4.6%; left atrial LS: 27.7±4.3% vs 41.0±11.5%; right atrial LS: 26.8±7.4% vs 44.0±7.9% and early pulsed wave to tissue Doppler mitral filling ratio (E/E’): 8.1±2.6 vs 5.8±1.3 were impaired in the ALCAPA population in comparison to the control group (p<0.01 for all comparisons). LV radial and circumferential strain did not differ between groups. Mean LS in the ALCAPA patients in the RCA region was -19.0±4.4%, while in the LCA region -13.8±7.3% (p<0.00001).ConclusionsDespite good clinical condition and normalized LV ejection fraction in ALCAPA patients after surgical repair in the long-term follow-up, the diastolic and longitudinal systolic function of all cardiac chambers remained impaired, especially in the LCA region. Lifelong surveillance of repaired ALCAPA patients is needed.
Highlights
Anomalous origin of the left coronary artery (LCA) from the pulmonary artery (ALCAPA), known as Bland-White-Garland syndrome, is a rare congenital heart disease, occurring approximately in 1 per 300.000 live births [1]
The aim of this study was to determine the incidence of myocardial dysfunction by means of tissue Doppler and speckle tracking echocardiography (STE) in the long-term observation of the ALCAPA patients operated in their childhood
Clinical data of the study population at the time of ALCAPA diagnosis is presented in S1 Table
Summary
Anomalous origin of the left coronary artery (LCA) from the pulmonary artery (ALCAPA), known as Bland-White-Garland syndrome, is a rare congenital heart disease, occurring approximately in 1 per 300.000 live births [1]. Retrograde flow in the LCA supplied from the right coronary artery (RCA) through natural collaterals to the low-pressure main pulmonary artery causes extensive myocardial ischemia. Ischemia occurs due to loss of perfusion pressure in the coronary arteries resulting from this specific steal syndrome. Most often it manifests in early childhood with symptoms of congestive heart failure or myocardial ischemia, and if left untreated the mortality reaches 90% in the first year of life. Retrograde flow from the right coronary artery (RCA) through natural collaterals to the low-pressure main pulmonary artery causes extensive ischemia. Limited data concerning the extent of permanent myocardial damage and functional recovery after surgical repair in the long-term follow-up is available.
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