Adult ALCAPA: from histological picture to clinical features.

BACKGROUND: Anomalous left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital coronary anomaly that results in high mortality if left untreated. Our aim was to extend our knowledge of the histological, angiographic, and clinical characteristics of ALCAPA in order to deepen our understanding of this rare entity. CASE PRESENTATION: We were involved in the assessment, treatment, and pathological evaluation of two adult ALCAPA patients who were rescued from ventricular fibrillation and then surgically treated to establish a dual coronary artery system. Histological studies indicated various chronic ischemic changes in the myocardium, patchy fibrosis, and severely thickened arteriolar walls in both ventricles. The first patient is alive and well 11.5 years after surgical correction without any implantable cardioverter defibrillator (ICD) activations. The second patient required re-do surgery 9 months after the initial operation but subsequently died. Histologically, chronic ischemic alteration of the myocardium and thickened arteriolar walls persisted even after surgical correction, and coronary angiography (CAG) showed an extremely slow flow phenomenon even after surgical correction in both patients. The average postoperative opacification rate in the first case was 7.36 + 1.12 (n = 2) in the RCA, 3.81 + 0.51 (n = 3) in the left anterior descending (LAD) artery, and 4.08 + 0.27 (n = 4) in the left circumflex (LCx) artery. The slow flow phenomenon may represent persistent high arteriolar resistance in both ventricles. CONCLUSIONS: Seldom reported or new findings in adult ALCAPA were identified in two cases. More frequent diagnosis of adult ALCAPA can be expected because of the widespread availability of resuscitation and more advanced diagnostic modalities. Accumulation of pathological and clinical findings and confirmation of the long-term follow-up results after treatment may contribute to expanding our knowledge of this rare entity and establishing optimal treatment.

A case of dual left anterior descending artery with myocardial infarction

The Left anterior descending artery (LAD) is a branch of the left main coronary artery which runs obliquely towards the apex of the heart in the anterior interventricular sulcus. Among all of the coronary arteries, the LAD artery has the most constant course. Amongst the anomalies of coronaries, the duplication or bifurcation of the LAD artery is infrequent. The classification of the bifurcation of the LAD has been extensively described in various reports with the widespread use of CT Angiography. We describe herein, an anomalous LAD detected on autopsy. This unusual case highlights the gross autopsy finding of Type-I anomalous dual LAD coronary artery in a young adult who died of Myocardial Infarction.

Myocardial function in patients with anomalous left coronary artery from the pulmonary artery syndrome: A long-term speckle tracking echocardiographic study.

BACKGROUND: Anomalous 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. AIM: Determination of the incidence of incipient myocardial dysfunction in ALCAPA patients in the long-term observation using tissue Doppler and speckle tracking echocardiography. METHODS AND RESULTS: Eighteen 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). CONCLUSIONS: Despite 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.