A 19-Year-Old Pregnant Woman With Pulmonary Hypertension With Progressive Dyspnea.

CASE PRESENTATION: A 19-year-old pregnant woman at week 32 of gestation was referred to our clinic with progressive shortness of breath for the further evaluation and treatment of high-risk pregnancy. Her complaints had been existing since her childhood. Two years prior to her admission, she had been diagnosed with heart failure with preserved ejection fraction due to cardiomyopathy and associated pulmonary hypertension. The patient had no family history of any cardiac disease. She had never smoked or drunk alcohol. Her clinical condition had deteriorated progressively with the pregnancy.

Early Outcomes of Transconal Repair of Transseptal Anomalous Left Coronary Artery From Right Sinus.

BACKGROUND: Anomalous aortic origin of the left coronary artery (AAOCLA) with an extended transseptal course behind the right ventricular outflow tract (RVOT) is a rare variant that poses challenges not addressed by current surgical techniques. We utilized a novel transconal approach in 7 consecutive patients. METHODS: A retrospective review was made of a prospectively collected database for consecutive patients undergoing transconal unroofing of transseptal AAOLCA. Surgical repair entails transection of the RVOT, unroofing the septal course of the AAOLCA, followed by RVOT extension with a rectangular strip of autologous pericardium. Preoperative characteristics, operative details, and postoperative course were abstracted. RESULTS: All 7 patients identified were symptomatic. Median age was 48 years (range, 12 to 62). The AAOLCA with transseptal course was confirmed by computed tomography angiography. Three patients had provocative testing demonstrating anterolateral ischemia. Four patients underwent cardiac catheterization with intravascular ultrasound and indexed fractional flow reserve, confirming flow-limiting lesion. Importantly, 3 patients had negative provocative noninvasive testing for ischemia. Median postoperative hospital length of stay was 6 days (range, 4 to 12). No mortality or major complications occurred during a median follow-up of 0.75 years. Postoperative evaluation demonstrated anatomically patent unroofed AAOLCA with improved indexed fractional flow reserve compared with preoperative (0.59 ± 0.16 vs 0.90 ± 0.03, P = .05). CONCLUSIONS: Complete unroofing of AAOLCA with transseptal course repaired with posterior extension of RVOT is an effective technique with excellent early outcome. Multimodality provocative testing is critical to evaluate these lesions as individual studies may be misleading. Intravascular ultrasound with indexed fractional flow reserve is clinically useful to confirm the hemodynamic significance of specific lesions.

Implantation of a Coronary Sinus Reducer to Treat Refractory Angina in a 38-Year-Old with an Anomalous Left Coronary Artery and No Revascularization Options.

A 38-year-old female with anomalous left coronary artery from the pulmonary artery presented with refractory angina (Canadian Cardiovascular Society [CCS] class 4). Having failed two previous internal mammary artery grafts to the left anterior descending artery and with no percutaneous revascularization options, she underwent coronary sinus reducer implantation, which improved her symptoms (CCS 0), quality of life, and corresponded to an improvement in ischemia on myocardial perfusion scanning. This case report describes an unusual case of refractory angina in the context of congenital heart disease, illustrates the benefit of this novel hourglass-shaped stent in improving ischemia, quality of life, depression and anxiety, and highlights the importance of managing these patients in multidisciplinary teams.

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.