Impact of Reverse Left Ventricular Remodeling on Outcomes of Patients with Anomalous Left Coronary Artery from the Pulmonary Artery after Surgical Correction.

The impact of reverse left ventricular remodeling (r-LVR) on clinical outcomes after surgical correction of anomalous left coronary artery from the pulmonary artery (ALCAPA) remains unclear. This study aims to examine the prognostic significance of r-LVR in patients with ALCAPA after surgery. We prospectively identified 61 patients undergoing surgical correction for ALCAPA; 54 patients had adequate echocardiographic image quality with quantitative biplane analysis performed both at baseline and at 30-day postoperative follow-up. Postoperative r-LVR was defined as a reduction of ≥ 10% in left ventricular end-diastolic volume index during follow-up. Cox proportional-hazards regression was used to investigate the independent association of r-LVR and all-cause mortality. Among 54 patients (age: 21.2 ± 7 months; 37% females), r-LVR occurred in 35 patients (64.8%) after surgery. Compared to patients with r-LVR, patients without r-LVR had significantly higher level of N-terminal pro B-type natriuretic peptide (NT-proBNP) [2176 (711, 4219) vs 998 (623, 2145) P < 0.001] and lower survival rate (47.3% vs 82.9%, HR = 5.72 [1.96 to 17.20], P < 0.001) at 1-year follow-up. NT-proBNP (OR = 2.27 [1.67 to 18.3], P = 0.02) was an independent predictor of r-LVR in multivariate analysis. Moreover, r-LVR was significantly associated with a lower rate of all-cause mortality (HR = 0.27 [0.08 to 0.98], P = 0.03) in multivariate analysis, even after adjustment for clinical and echocardiographic variables. R-LVR occurred in more than half of patients with ALCAPA undergoing surgical correction and it was associated with better clinical outcomes. NT-proBNP is an independent predictor of r-LVR.

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.

Effective myocardial perfusion and concomitant haemodynamic status determine the clinical diversity of anomalous left coronary artery from the pulmonary artery.

BACKGROUND: Anomalous left coronary artery from the pulmonary artery is a rare congenital heart disease (CHD) with diverse clinical presentations despite the same anatomy. Factors determining this heterogeneous presentation are not well understood. METHOD AND RESULTS: We retrospectively investigated 14 patients (12 females) who underwent surgical repair of anomalous left coronary artery from the pulmonary artery. These patients were divided into three groups based upon the severity of initial presentation: (1) severe, life-threatening condition (n = 5), (2) mild-to-moderate distress (n = 6), and (3) asymptomatic (n = 3). All patients presented with left ventricular dilation and retrograde flow in left coronary artery by echocardiogram. Eight patients in (1) and (2) presented with severe left ventricular dysfunction. All but one showed abnormal ECG consistent with myocardial ischemia or infarction. Asymptomatic patients had preserved left ventricular systolic function despite ischemic findings on ECG. In 13 patients after surgical repair, all but one normalised left ventricular geometry and systolic function, suggesting nearly full myocardial recovery upon improvement of myocardial perfusion; 8 patients had residual echogenic papillary muscle with variable degree of mitral regurgitation. CONCLUSIONS: Evidence of myocardial ischemic injury was present in all patients with anomalous left coronary artery from the pulmonary artery regardless of their initial presentation. Retrograde flow in left coronary artery, implying collateral vessel development from right coronary artery to left coronary artery, was noted in all patients, yet only few patients had preserved systolic function at the time of diagnosis. The balance between effective myocardial perfusion and a deleterious fistulous flow provided by these collaterals and the simultaneous haemodynamic status are what determine the clinical diversity of anomalous left coronary artery from the pulmonary artery.

Oblique coronary artery prolongation in anomalous left coronary artery arising from the pulmonary artery: A case series.

BACKGROUND: Anomalous left coronary artery (LMCA) arising from the pulmonary artery (ALCAPA) is a life-threatening congenital birth defect. Prompt surgical correction by reimplantation of the LMCA into the aorta is the treatment of choice, however, unfavorable LCA location or length can make direct reimplantation difficult or even impossible without causing significant stretching or tension on the LMCA. This tension can lead to stenosis of the artery and coronary ischemia and must be avoided if possible. METHODS: A chart review from January 2010 to December 2018 of patients who had ALCAPA repair. Patients were excluded if direct reimplantation was possible. Herein, we describe four cases in which remote LMCA position necessitated the creation of a tubular prolongation of the LMCA. RESULTS: A generous coronary button from the pulmonary artery along with the oblique flap of aorta served as the prolongation of the left main coronary artery. This allowed for a tension-free anastomosis using native tissue with the ability to grow with the patient. The pulmonary artery and the aorta were then patched using pericardial tissue. All four patients had improvement in left ventricular function and degree of mitral regurgitation within weeks of surgery. No patient required extracorporeal membrane oxygenation support. Subsequent long-term angiographic follow-up has revealed excellent durability and patency of these coronary conduits. CONCLUSIONS: The variable nature of ALCAPA requires modification of the surgical approach on a patient by patient basis, and the above described technique adds an alternate and reliable long-term treatment option.

Repeat Repair After Translocation of an Anomalous Left Coronary Artery.

We repaired a left anomalous aortic origin of coronary artery using coronary ostium translocation/reimplantation. Repeat repair was required due to suboptimal flow pattern in postoperative transesophageal echocardiography, which was confirmed with coronary angiography. We emphasize the importance of early recognition, diagnosis, and intervention to deal with a problem with the coronary repair.

Anomalous Left Coronary Artery From the Pulmonary Artery With Ventricular Septal Defect and Double Aortic Arch: A Unique Case Report.

Anomalous left coronary artery from the pulmonary artery (ALCAPA) is an uncommon life-threatening congenital heart defect that occurs mostly as an isolated lesion. It is very rarely associated with other cardiac abnormalities. In this report, we present a case of an infant who successfully underwent complete surgical repair of a rare triad of congenital heart defects, including ALCAPA, ventricular septal defect (VSD), and double aortic arch (DAA). To the best of our knowledge, this is only the second case of ALCAPA diagnosis associated with VSD and DAA that has been described in the literature.

Intermediate Outcomes After Repair of Anomalous Left Coronary Artery From the Pulmonary Artery.

BACKGROUND: Multicenter studies on infants with anomalous left coronary artery from the pulmonary artery (ALCAPA) are lacking. We report the intermediate-term outcomes after ALCAPA repair in a multicenter cohort and identify risk factors for reintervention or death after discharge. METHODS: We retrospectively reviewed infants under 1 year of age who underwent ALCAPA repair from January 2009 to March 2018 at 21 US centers. The primary composite outcome was freedom from reintervention or death after discharge. We used the Kaplan-Meier survival analysis to examine freedom from reintervention or death and the Cox proportional hazard analysis to identify risk factors for this composite outcome. RESULTS: One hundred seventy-seven infants underwent ALCAPA repair; 170 (97%) survived to hospital discharge without transplantation. Twenty-three patients were lost to follow-up. The median duration of follow-up in the remaining 147 patients was 3.8 years (25%, 75%: 1.9 years, 6.0 years). Echocardiographic data were available at ∼3 years after discharge in 98 patients. Left ventricular function was normal in 96 patients (98%), whereas 26 patients (27%) had greater than mild mitral valve regurgitation. Sixteen patients (11%) underwent 20 reinterventions with 1 late death. Patients undergoing the Takeuchi procedure or atypical repairs (hazard ratio, 8.0; 95% confidence interval, 2.1-30.0) or with moderate or greater mitral regurgitation on discharge echocardiogram (hazard ratio, 3.4; 95% confidence interval, 1.2-9.1) were at increased risk for reintervention. CONCLUSIONS: Intermediate-term outcomes after ALCAPA repair in infants are favorable. Persistent left ventricular dysfunction and reinterventions were uncommon, and mortality was rare. Patients who required atypical surgical repair or had moderate or greater mitral regurgitation at discharge warrant closer follow-up.

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.

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.

Surgical outcomes for anomalous left coronary artery from the pulmonary artery: Influence of late presentation.

OBJECTIVES: Although surgical outcomes for anomalous left coronary artery from the pulmonary artery (ALCAPA) are excellent in the modern era with the coronary reimplantantion technique, mortality remains high in Chinese population. This study was undertaken to review the surgical management for ALCAPA in our center and assess the midterm outcomes. METHODS: This was a retrospective review of 105 patients who underwent surgical repair for ALCAPA from January 2008 to January 2018. RESULTS: The diagnosis of ALCAPA was not made before referred to our hospital in 31 patients (30%). Median age at repair was 7.6 months (interquartile range, 4.3-25.4 months). Mean preoperative left ventricular ejection fraction was 47.3% ± 16.9%. Concomitant mitral intervention was performed in 52 patients (50%). Mechanical circulatory support was used in 15 patients (14%). Early mortality was 14%. Classification and regression tree analysis identified 3 risk groups for early mortality, among which patients with preoperative left ventricular ejection fraction ≤41.6% and age at repair >127 days were the most likely to suffer from mortality. Using nonlinear mixed-effect model to assess the time course for postoperative left ventricular ejection fraction, patients of this group also needed significantly longer time for recovery of left ventricular function after surgery (P < .0001). CONCLUSIONS: Late referral of patients with ALCAPA is not uncommon in our center. Older age at repair in patients with preoperative left ventricular dysfunction is the main reason for higher early mortality, and is also associated with longer time to normalization of left ventricular function after surgery.

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.

Anomalous Left Coronary Artery From The Pulmonary Artery (ALCAPA) as a Cause of Heart Failure.

BACKGROUND Anomalous left coronary artery from the pulmonary artery (ALCAPA) is a rare anomaly. When present it can result in failure to thrive and congestive heart failure. CASE REPORT We present the case of a 6-month old female whose presentation was one of failure to thrive. Point of care ultrasound and electrocardiogram (ECG) were used to diagnose heart failure with consideration of ALCAPA. These tools helped to expedite transfer to a tertiary care center for definitive therapy. CONCLUSIONS Although a rare anomaly, ALCAPA induced heart failure can be quickly identified on bedside ultrasound. Together with ECG findings, the Emergency Physician can expedite the diagnosis and proper disposition.

Surgical treatment of anomalous left main coronary artery with an intraconal course.

OBJECTIVE: Anomalous left main coronary artery (LMCA) with an intraconal course is a relatively rare form of anomalous aortic origin of a coronary artery (AAOCA) from the wrong sinus of Valsalva. There is currently a paucity of information regarding this entity. The purpose of this article is to review our surgical experience with repair of anomalous LMCA with an intraconal course. METHODS: This was a retrospective review of 12 patients with an anomalous LMCA and an intraconal course who underwent surgical repair. The median age at surgery was 15 years (range 2-47). The seven oldest patients all had preoperative symptoms of exertional chest pain and one also had exertional syncope. The five youngest patients had no preoperative symptoms. One of these patients had a hemodynamically significant ventricular septal defect and one patient was the sibling of a patient who had undergone repair of AAOCA. RESULTS: The 12 patients underwent surgical repair including unroofing of the myocardial bridge overlying the intraconal LMCA and a LeCompte procedure. There was no early or late mortality and there were no significant complications. All 12 patients have resumed normal, unrestricted activities. CONCLUSIONS: Twelve patients with an anomalous LMCA and intraconal course presented to our institution for treatment. Surgical repair was performed successfully in all 12, with resolution of symptoms in the 7 patients who were symptomatic preoperatively. These results suggest that the surgical treatment is safe and efficacious in patients with an anomalous LMCA and intraconal course.

Transconal Unroofing of Anomalous Left Main Coronary Artery From Right Sinus With Trans-septal Course.

Anomalous aortic origin of the left main coronary artery arising from the right coronary sinus with a trans-septal course are rare and pose surgical challenges not addressed by current techniques. Coronary artery bypass grafting, although a plausible solution, has an unacceptably high incidence of graft failure due to competitive flow. We describe a novel transconal approach to effectively unroof the trans-septal left main coronary artery.

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.