ABSTRACT
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.
Subject(s)
Anomalous Left Coronary Artery , Cardiomyopathies , Cardiovascular Surgical Procedures/methods , Heart Failure , Hypertension, Pulmonary , Postnatal Care/methods , Pregnancy Complications, Cardiovascular , Adult , Anomalous Left Coronary Artery/complications , Anomalous Left Coronary Artery/diagnostic imaging , Anomalous Left Coronary Artery/physiopathology , Anomalous Left Coronary Artery/surgery , Cardiac Catheterization/methods , Cardiomyopathies/complications , Cardiomyopathies/diagnosis , Cesarean Section/methods , Computed Tomography Angiography/methods , Dyspnea/diagnosis , Dyspnea/etiology , Echocardiography/methods , Female , Heart Failure/complications , Heart Failure/diagnosis , Heart Failure/physiopathology , Humans , Hypertension, Pulmonary/diagnosis , Hypertension, Pulmonary/etiology , Hypertension, Pulmonary/physiopathology , Pregnancy , Pregnancy Complications, Cardiovascular/diagnosis , Pregnancy Complications, Cardiovascular/etiology , Pregnancy Complications, Cardiovascular/physiopathology , Pregnancy Complications, Cardiovascular/therapy , Pregnancy, High-Risk , Stroke Volume , Treatment OutcomeABSTRACT
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.
Subject(s)
Anomalous Left Coronary Artery/surgery , Cardiac Catheterization/methods , Coronary Sinus/abnormalities , Heart Septum/surgery , Vascular Surgical Procedures/methods , Adolescent , Adult , Anomalous Left Coronary Artery/diagnosis , Anomalous Left Coronary Artery/physiopathology , Child , Computed Tomography Angiography , Coronary Sinus/diagnostic imaging , Female , Follow-Up Studies , Fractional Flow Reserve, Myocardial/physiology , Humans , Male , Middle Aged , Retrospective Studies , Time Factors , Treatment Outcome , Young AdultABSTRACT
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.
Subject(s)
Angina Pectoris/surgery , Anomalous Left Coronary Artery/surgery , Coronary Sinus/surgery , Vascular Surgical Procedures/instrumentation , Adult , Angina Pectoris/diagnostic imaging , Angina Pectoris/physiopathology , Anomalous Left Coronary Artery/diagnostic imaging , Anomalous Left Coronary Artery/physiopathology , Coronary Angiography , Coronary Sinus/diagnostic imaging , Coronary Sinus/physiopathology , Female , Humans , Quality of Life , Stents , Treatment Outcome , Vascular Surgical Procedures/adverse effectsSubject(s)
Anomalous Left Coronary Artery/diagnostic imaging , Computed Tomography Angiography , Coronary Angiography , Coronary Sinus/diagnostic imaging , Coronary Stenosis/diagnostic imaging , Aged , Anomalous Left Coronary Artery/physiopathology , Anomalous Left Coronary Artery/surgery , Coronary Artery Bypass , Coronary Sinus/abnormalities , Coronary Sinus/physiopathology , Coronary Sinus/surgery , Coronary Stenosis/physiopathology , Coronary Stenosis/surgery , Humans , Male , Myocardial Perfusion Imaging , Predictive Value of TestsSubject(s)
Anomalous Left Coronary Artery/diagnostic imaging , Computed Tomography Angiography , Coronary Angiography , Coronary Sinus/diagnostic imaging , Anomalous Left Coronary Artery/physiopathology , Cardiac-Gated Imaging Techniques , Coronary Sinus/physiopathology , Electrocardiography , Humans , Magnetic Resonance Imaging , Male , Middle Aged , Predictive Value of TestsSubject(s)
Abnormalities, Multiple/diagnostic imaging , Anomalous Left Coronary Artery/diagnostic imaging , Computed Tomography Angiography , Coronary Angiography , Coronary Vessels/diagnostic imaging , Multidetector Computed Tomography , Pulmonary Artery/diagnostic imaging , Vascular Malformations/diagnostic imaging , Abnormalities, Multiple/physiopathology , Abnormalities, Multiple/surgery , Anomalous Left Coronary Artery/physiopathology , Anomalous Left Coronary Artery/surgery , Coronary Vessels/physiopathology , Coronary Vessels/surgery , Humans , Infant , Predictive Value of Tests , Pulmonary Artery/abnormalities , Pulmonary Artery/physiopathology , Pulmonary Artery/surgery , Vascular Malformations/physiopathology , Vascular Malformations/surgerySubject(s)
Abnormalities, Multiple/diagnostic imaging , Anomalous Left Coronary Artery/diagnostic imaging , Computed Tomography Angiography , Coronary Angiography , Coronary Sinus/diagnostic imaging , Pulmonary Artery/diagnostic imaging , Vascular Malformations/diagnostic imaging , Abnormalities, Multiple/physiopathology , Adult , Anomalous Left Coronary Artery/physiopathology , Collateral Circulation , Coronary Circulation , Coronary Sinus/abnormalities , Coronary Sinus/physiopathology , Humans , Male , Predictive Value of Tests , Pulmonary Artery/abnormalities , Pulmonary Artery/physiopathology , Pulmonary Circulation , Vascular Malformations/physiopathologySubject(s)
Anomalous Left Coronary Artery/diagnostic imaging , Computed Tomography Angiography , Coronary Angiography , Myocardial Bridging/diagnostic imaging , Myocardial Ischemia/diagnostic imaging , Anomalous Left Coronary Artery/complications , Anomalous Left Coronary Artery/physiopathology , Anomalous Left Coronary Artery/therapy , Conservative Treatment , Female , Humans , Middle Aged , Myocardial Bridging/complications , Myocardial Bridging/physiopathology , Myocardial Bridging/therapy , Myocardial Ischemia/etiology , Myocardial Ischemia/physiopathology , Myocardial Ischemia/therapy , Predictive Value of TestsSubject(s)
Anomalous Left Coronary Artery/complications , Hemodynamics , Pulmonary Artery/abnormalities , Ventricular Dysfunction, Left/etiology , Ventricular Function, Left , Anomalous Left Coronary Artery/diagnostic imaging , Anomalous Left Coronary Artery/physiopathology , Anomalous Left Coronary Artery/surgery , Coronary Artery Bypass , Female , Humans , Ligation , Middle Aged , Pulmonary Artery/diagnostic imaging , Pulmonary Artery/physiopathology , Pulmonary Artery/surgery , Recovery of Function , Saphenous Vein/transplantation , Treatment Outcome , Ventricular Dysfunction, Left/diagnostic imaging , Ventricular Dysfunction, Left/physiopathologyABSTRACT
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.