Diagnosis and Surgical Outcomes of Patients with Anomalous Left Coronary Artery from the Pulmonary Artery: A Single Taiwanese Medical Center Experience.

BACKGROUND: Anomalous left coronary artery from the pulmonary artery (ALCAPA), a very rare congenital cardiac anomaly, is associated with a high mortality rate among infants who are not diagnosed or treated in a timely manner. Surgical intervention with the reconstruction for a two-coronary-system circulation is the main treatment; however, there have been very few reported cases from Taiwan. In this study, we aim to describe the clinical manifestations, diagnostic methods, surgery types, and surgical outcomes in patients with ALCAPA from a single Taiwanese medical center. METHODS: We retrospectively reviewed patients diagnosed with ALCAPA who underwent surgery at our institution between January 2001 and October 2018. Clinical presentations, noninvasive and invasive study results, surgical methods, and postoperative follow-up results were assessed from medical records. Moreover, literature on this particular cardiovascular anomaly was reviewed. RESULTS: The study included 6 patients (5 children and 1 adult). The diagnosis was confirmed using cardiac catheterization and coronary angiography in four patients and only echocardiography in two patients. All patients underwent surgical correction and survived. Four patients showed improvements in left ventricular function and mitral regurgitation (MR). CONCLUSION: Early diagnosis and timely surgical intervention could avoid mortality regardless of the method of operation. ALCAPA can be definitively diagnosed using noninvasive echocardiography. Both left ventricular systolic function and mitral insufficiency could improve after the surgical intervention in pediatric patients. Repair or replacement of the mitral valve could be reserved for persistent MR complicated with congestive heart failure, particularly in patients who received the initial operation beyond infancy.

Transthoracic echocardiography features of adult-type anomalous left coronary artery from the pulmonary artery before and after surgery: highlights from observational study in a single center of China.

To review the imaging characteristics of adult-type anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) and evaluate the post-operative echocardiographic features. The transthoracic echocardiography (TTE) records and invasive coronary angiography (ICA), coronary computed tomography angiography (CTA) or operative findings of thirty adult patients with final diagnosis of ALCAPA were reviewed at our center. The diagnostic reliability of TTE was determined by comparing its results with that of ICA/CTA, and the echocardiographic features of the patients during early visit and post-operative follow-up were summarized. The coincidence rate of TTE and ICA/CTA diagnosing ALCAPA was 96.3% (26/27), and one case was misdiagnosed as coronary artery fistula. All patients showed abnormal left coronary ostium arising from the pulmonary trunk with retrograde coronary artery flow, 20 patients had enlarged right coronary artery (RCA), and 20 showed abundant inter-coronary septal coronary collaterals. Left ventricle (LV) abnormalities such as wall thinning, abnormal wall motion, papillary muscle fibrosis, mitral valve (MV) prolapse and moderate-severe mitral regurgitation (MR) were respectively observed in 4, 7, 10, 8 and 10 patients. Three patients showed ventricular septal defect, patent foramen ovale and tetralogy of Fallot respectively. Post-operative data was available for 12 patients, and showed that the diameter of RCA, size of left atrium (LA) and LV were decreased after surgery compared to the respective pre-operative values. Furthermore, inter-coronary septal coronary collaterals lessened in 8 of these patients. Although 6 patients showed residual mild MR, moderate or severe MR was not observed, and the ejection fraction (EF) also showed no obvious changes before and after surgery. TTE is a non-invasive diagnostic tool for adult-type ALCAPA, and can indicate abnormal coronary origin, collateral arteries and other associated malformations. Some of the structural and hemodynamic parameters of adult-type ALCAPA were improved after surgery.

Anomalous origin of the left coronary artery from the pulmonary artery: A surgical certainty.

This study aims to assess the clinical presentation and the outcomes of a surgical correction of an anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA). A retrospective review was carried out on the charts of six patients admitted for ALCAPA surgery at Aga Khan University Hospital, Karachi from March 2017 to May 2018.Dyspnoea, palpitation, poor feeding, fatiguability, pallor and a murmur of mitral regurgitation were the main presenting features. The pre-operative median left ventricular ejection fraction (LVEF) was 64%. Coronary reimplantation was performed in all the patients with a mitral valve repair being done in only one patient. The mea n LV EF was 66 .3%,p ost- ope rativel y. Mitra l regurgitation (MR) improved in patients post-operatively with trace in 2 patients and mild MR in one. Surgical correction by coronary re-implantation yields favourable outcomes in ALCAPA and significantly reduces the morbidity and mortality rates associated with the disease.

A case of robotic assisted percutaneous coronary intervention of the left main coronary artery in a patient with very late baffle stenosis after surgical correction of anomalous left coronary artery from the pulmonary artery.

A 34-year-old woman with history of surgical correction (Takeuchi procedure) of anomalous left coronary artery from the pulmonary artery (ALCAPA) presented with reduced left ventricular ejection fraction of 48% and severe ischemia quantified as 21% by stress Positron Emission Tomography (PET) scan. A coronary angiogram revealed ostial 90% stenosis of the left main coronary artery (LMCA). A guidewire (Sion Blue, Asahi Intecc USA, Inc., Santa Ana, CA) was navigated robotically and after pre-dilation with 3.5 × 15 mm cutting balloon, the lesion length was measured by marking the distal end of the lesion with the balloon marker and withdrawing back robotically to the ostium of the LMCA. A 3.5 × 16 mm drug-eluting stent was deployed robotically after intravascular ultrasound (IVUS) with good results. The main advantage of robotic percutaneous coronary intervention includes the precise measurement and positioning of the stent. Since the guide catheter and balloon can be adjusted without guide catheter and device interaction, precise placement of stent is possible by advancing the device distal to the lesion, positioning the guide catheter just proximal to the proximal edge of the stent and pulling the guidecatheter and device back as a unit. Final IVUS after post-dilation with 4.0 noncompliant and 5.0 compliant balloon revealed precise placement at the ostium and full stent expansion.

Mid-Term Outcome for Anomalous Origin of the Left Coronary Artery From the Pulmonary Artery.

BACKGROUND: This study is a single-centre experience with surgical intervention of anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA). METHODS: A retrospective review was performed of patients who underwent ALCAPA repair between November 1991 and October 2017. RESULTS: A total of 138 patients with a median age of 36 months underwent ALCAPA repair. The median follow-up period was 3.5 years. Overall, 114 (82.6%) patients received coronary reimplantation, 21 (15.2%) underwent Takeuchi repair, and three (2.1%) were treated by closure of the ALCAPA plus coronary artery bypass graft. Concomitant mitral repair was performed in 50 patients with moderate-to-severe mitral regurgitation (MR). Three (2.1%) patients died in hospital. Three (2.1%) patients needed extracorporeal membrane oxygenation (ECMO) support. Seven (5.0%) patients needed delayed sternal closure. In patients with decreased preoperative left ventricular ejection fraction (LVEF) (<50%), the LVEF significantly improved from 25% to 33% at discharge (p = 0.001). The percentage of more than moderately significant MR decreased from 36.2% to 5.2% (p = 0.02). There was no late mortality. Two patients needed reoperation because of baffle leaks after Takeuchi repair. Three patients had severe MR. All patients had normal LVEF at last follow-up. The median normalisation time of left ventricular function was 6 months. CONCLUSIONS: Short-term and mid-term outcomes of surgical treatment for ALCAPA patients were excellent. The concomitant mitral valve repair did not increase the perioperative risk but had satisfactory mid-term outcomes. Normalisation of left ventricular function is expected within the first 6 months.

A patient with anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA syndrome) and 13 live births.

Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) or Bland-White-Garland syndrome is a rarely seen congenital anomaly. Adult and infantile types are defined according to the degree of collateral development between the left coronary artery (LCA) and right coronary artery (RCA). If left untreated, ALCAPA has a 90% mortality rate in the first year of life, primarily due to myocardial ischaemia and heart failure. The degree of collateral development and the related LCA perfusion in ALCAPA syndrome determine the occurrence of symptoms. Herein, we present a case of a female patient who had previously, without any symptoms, given live birth to 13 babies. She had been experiencing exertional angina, which started long after the delivery of her 13th child. Since our patient had well-developed collaterals to the LCA, she was asymptomatic and able to give birth to the children via the vaginal route without any problems. Having well-formed collateral vessels between the RCA and LCA may prevent patients from developing symptoms, and even stressful conditions such as pregnancy may be tolerable.

Oiigen anomalo de la arteria coronaiia derecha de la arteiia pulmonar en un lactante con soplo cardiaco: A proposito de un caso / Anomalous origin of the right coronary artery from the pulmonary artery in an infant with a heart murmur: Case report

El origen anómalo de la arteria coronaria derecha de la arteria pulmonar es una entidad rara con una incidencia del 0,002 % y potencialmente grave, que puede causar muerte súbita. El diagnóstico frecuentemente es incidental, secundario a evaluaciones por soplos cardíacos a diferencia del origen anómalo de la arteria coronaria izquierda de la arteria pulmonar, reconocido por presentar isquemia miocárdica e insuficiencia cardíaca. Se reporta el caso de una niña de 6 meses evaluada por un soplo y derivada por sospecha de fístula coronaria. La evaluación mediante ecocardiograma doppler color, cateterismo cardíaco y angiotomografía mostró la presencia de una comunicación interauricular ostium secundum pequeña y origen anómalo de la arteria coronaria derecha de la arteria pulmonar. A los 11 meses, se realizó una cirugía de reimplante de la arteria coronaria derecha en la arteria aorta y el cierre de la comunicación interauricular.

The anomalous origin of the right coronary artery from the pulmonary artery (ARCAPA) is a rare entity that has an incidence of 0.002 % and can potentially cause sudden death. Unlike to the anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA), usually presented with myocardial ischemia and heart failure; the diagnosis of ARCAPA is often incidental during evaluation of a heart murmur. We report a case of a 6 months old female patient referred to us with a cardiac murmur and a suspicion of coronary fistula. For a proper diagnostic assessment an echocardiogram, a computed tomography angiography and a cardiac catheterization were requested. A small ostium secundum-type atrial septal defect (ASD) and an ARCAPA were revealed. At 11 months old, the patient was scheduled for corrective cardiovascular surgery. The aim of this report is to describe a low incidence and potentially fatal disease.

Anomalous origin of the right coronary artery from the pulmonary artery in an infant with a heart murmur: Case report. / Oiigen anomalo de la arteria coronaiia derecha de la arteiia pulmonar en un lactante con soplo cardiaco: A proposito de un caso.

The anomalous origin of the right coronary artery from the pulmonary artery (ARCAPA) is a rare entity that has an incidence of 0.002 % and can potentially cause sudden death. Unlike to the anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA), usually presented with myocardial ischemia and heart failure; the diagnosis of ARCAPA is often incidental during evaluation of a heart murmur. We report a case of a 6 months old female patient referred to us with a cardiac murmur and a suspicion of coronary fistula. For a proper diagnostic assessment an echocardiogram, a computed tomography angiography and a cardiac catheterization were requested. A small ostium secundum-type atrial septal defect (ASD) and an ARCAPA were revealed. At 11 months old, the patient was scheduled for corrective cardiovascular surgery. The aim of this report is to describe a low incidence and potentially fatal disease.

El origen anómalo de la arteria coronaria derecha de la arteria pulmonar es una entidad rara con una incidencia del 0,002 % y potencialmente grave, que puede causar muerte súbita. El diagnóstico frecuentemente es incidental, secundario a evaluaciones por soplos cardíacos a diferencia del origen anómalo de la arteria coronaria izquierda de la arteria pulmonar, reconocido por presentar isquemia miocárdica e insuficiencia cardíaca. Se reporta el caso de una niña de 6 meses evaluada por un soplo y derivada por sospecha de fístula coronaria. La evaluación mediante ecocardiograma doppler color, cateterismo cardíaco y angiotomografía mostró la presencia de una comunicación interauricular ostium secundum pequeña y origen anómalo de la arteria coronaria derecha de la arteria pulmonar. A los 11 meses, se realizó una cirugía de reimplante de la arteria coronaria derecha en la arteria aorta y el cierre de la comunicación interauricular.

A Novel Aortic Reconstruction for Anomalous Left Coronary Arising From the Right Pulmonary Artery in Hypoplastic Left Heart Syndrome: Successful Surgical Treatment.

An anomalous left coronary arising from the pulmonary artery (ALCAPA) in hypoplastic left heart syndrome (HLHS) is a very rare congenital malformation. The Norwood procedure and simultaneous direct reimplantation of the left coronary artery (LCA) to the ascending aorta have been previously used for treatment of ALCAPA, although mortality was very high, because it is difficult to reimplant the LCA to the neoaortic root without distortion or tension. We chose the modified Norwood procedure without reimplantation of the LCA to the neoaortic root, and 4-month-old male infant survived the operation and waiting Fontan procedure.

Cardiac magnetic resonance imaging, myocardial scar and coronary flow pattern in anomalous origin of left coronary artery from the pulmonary artery.

BACKGROUND: Anomalous origin of left coronary artery from pulmonary artery (ALCAPA) is a very rare congenital heart defect characterized by myocardial ischemia and ultimately scaring. The scar burden will determine eventual recovery of left ventricular function after corrective surgery. MATERIAL METHOD: All patients with proven diagnosis of ALCAPA and who underwent treatment at present centre were included. Detail echocardiography and cardiac magnetic resonance imaging (CMR) (delayed Gadolinium enhancement) was performed before and after surgery. RESULTS: There were 4 patients (3 females, age group 3 months to 3 yr, follow up 6 months to 20 months.) There was no peri operative mortality. All patients had significant improvement in symptom class and LVEF (increase of more than 10%) when evaluated at last follow up. Three patients had pre operative CMR and 3 post operative CMR. All patients had improvement in post operative LVEF, but >50% was observed only in one patient who had less than half thickness delayed gadolinium enhancement. The right coronary flow pattern were unique to disease. The left coronary flow pattern were had significant variation and could predict extent of scared myocardium. CONCLUSION: Ischemia in ALCAPA can lead to myocardial scarring even in early infancy. The recovery in left ventricular function is a closely related to scar burden. Coronary flow patterns are unique and give useful insight into disease process and natural history.

The outcome of mitral regurgitation after the repair of anomalous left coronary artery from the pulmonary artery in infants and older children.

OBJECTIVES: Mitral regurgitation (MR) in the majority of infants with an anomalous left coronary artery from the pulmonary artery (ALCAPA) has been shown to improve without concomitant mitral valve repair. However, the outcome of MR in older children with ALCAPA is unclear. The purpose of this study was to compare the outcome of MR following the ALCAPA repair in infants and older children. METHODS: Forty-six patients (34 were younger than 1 year and 12 were aged 1-12 years) underwent the repair for ALCAPA in our institution from June 2006 to June 2016. Baseline and follow-up data were collected from records, and the latest echocardiogram was performed for all surviving patients from June 2017 to August 2017. RESULTS: Of the 34 infants, 82% had moderate-to-severe ventricular dysfunction, and 47% had significant MR at presentation. Fifty-seven percent of the older age group had MR, though none of them had ventricular dysfunction. Two infants and 3 older children underwent concomitant mitral valve repair. There were 2 early deaths in the infantile group. On follow-up, ventricular function normalized in 88% of infants; 12.5% of the infants had moderate-to-severe MR on follow-up, and 50% of the older children had moderate-to-severe MR on follow-up. CONCLUSIONS: The ALCAPA repair has excellent survival outcomes in all age groups. MR improves in the majority of infants with revascularization alone. MR worsens or persists in a significant number of older children following the ALCAPA repair, and they are more likely to require reinterventions for the mitral valve on follow-up.

Nicorandil was an Effective Treatment Option for a Patient with Bland-White-Garland Syndrome.

A 75-year-old woman who had previously been diagnosed with Bland-White-Garland syndrome was admitted to our hospital for acute decompensated heart failure (ADHF). Following her recovery from ADHF, pharmacologic stress myocardial scintigraphy revealed myocardial ischemia in the basal anterior area of the left ventricle. Moreover, myocardial scintigraphy showed the improvement of the myocardial ischemia after 6 months of nicorandil administration.

Misdiagnosed anomalous left coronary artery from the pulmonary artery as endocardial fibroelastosis in infancy: A case series.

INTRODUCTION: Anomalous left coronary artery from the pulmonary artery (ALCAPA) is a rare but severe congenital cardiac malformation. The prognosis mainly depends on the early and accurate diagnosis and treatment. However, without a typical and specific clinical manifestation in early stage, ALCAPA has a higher rate of false initial diagnosis. DIAGNOSTIC AND THERAPEUTIC PROCEDURE: Three infants with impaired left ventricle (LV) function, LV enlargement, mitral valve regurgitation (MR), and LV endocardium thickness were initially diagnosed as endocardial fibroelastosis (EFE). Due to the treatment effectiveness with prednisone acetate and digoxin, abnormal Q waves with T inversion, and dilated right coronary artery (RCA), the diagnosis of ALCAPA was suspected. Lastly, cardiac angiography confirmed the diagnosis. All of them were transferred to the cardiac surgery department and received a successful surgical repair. The follow-up results showed that abnormal Q waves with T waves inversion on electrocardiogram gradually regressed and disappeared, LV ejection fraction and LV dilation returned to a normal range after surgery, with alleviation of MR. Besides, endocardial thickness secondary to ischemia also returned to normal. CONCLUSION: ALCAPA should be suspected when confronted with patients with left heart enlargement, impaired left ventricular function, and signs of myocardial ischemia, particularly in infancy. EFE is an important differential diagnosis and may also arise as a result of ALCAPA. Abnormal Q waves with T waves inversion, particularly in avL, dilated RCA and increased ratio of RCA/AO are important differential key points for the identification of ALCAPA and EFE. Awareness of this condition is essential for prompt recognition and referral to a tertiary cardiac center to enable early surgical intervention and improved prognosis for these children.

Outcomes from anomalous origin of the left coronary artery from the pulmonary artery repair: Long-term complications in relation to residual myocardial abnormalities.

BACKGROUND: Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital coronary artery irregularity. This study aimed to clarify the long-term postoperative outcomes in ALCAPA patients, and to compare the infantile and adult types. METHODS: We retrospectively analyzed the clinical data from 33 patients with ALCAPA who underwent surgical repairs after 1980. The patients were grouped based on whether presentation occurred before (infantile type: n=14) or after (adult type: n=19) 1 year of age. RESULTS: The mean follow-up duration was 16 years. Preoperatively, the infantile type had greater impairment of the left ventricle ejection fraction (LVEF) (45±15%) compared with the adult type (59±10%) (p<0.01). Coronary revascularization significantly improved the postoperative LVEF (67±5%) (p<0.01) in the patients with the infantile type. The postoperative LVEF did not change in the adult type. The mitral regurgitation (MR) severity improved postoperatively, but the between-group difference was not significant. Postoperatively, none of the patients with the infantile type and 37% of the patients with the adult type had left ventricular asynergy (p=0.01), and both groups showed postoperative perfusion defects (79% vs 95%, p=0.29). Compared with the infantile type, the adult type had a significant prognostic value for composite cardiovascular events that comprised cardiac death, arrhythmias, MR deterioration, and hospitalization as a consequence of heart failure (p=0.04). CONCLUSIONS: Most patients showed favorable clinical outcomes postoperatively, but myocardial damage remained long after surgery and cardiovascular events occurred postoperatively. Hence, meticulous long-term follow-up is warranted.

Left Ventricular Mechanics in Patients with Abnormal Origin of the Left Main Coronary Artery from the Pulmonary Trunk Late after Successful Repair.

OBJECTIVE: Our aim was to evaluate left ventricular (LV) mechanics by using speckle tracking echocardiography (STE) in asymptomatic patients with abnormal origin of the left main coronary artery from the pulmonary trunk (ALCAPA), late after successful repair, in the presence of LV ejection fraction (EF) >50%. METHODS: We studied 30 ALCAPA patients (median age 4 years, range 1-25 years, NYHA class I, LVEF >50%) and 16 healthy age- and sex-matched controls (median age 5 years, range 1-25 years). All underwent standard echocardiographic evaluation and STE. RESULTS: LV dimensions and LVEF (63.6 ± 8.2% vs. 64.1 ± 5.1%, p = 0.826) were not different between patients and controls. Diastolic parameters were significantly abnormal in our patients versus controls (E/e' average: 11.9 ± 5.8 vs. 6.6 ± 3.0, p = 0.0014). Global LV longitudinal strain was significantly lower in ALCAPA patients versus controls (-17.6 ± 3.5% vs. -23.4 ± 3.1%, p < 0.0001). LV torsion (9.1 ± 4.9° vs. 11.9 ± 3.3°, p = 0.046) was significantly impaired in ALCAPA patients. CONCLUSIONS: After successful repair in asymptomatic ALCAPA patients, despite an LVEF >50%, diastolic function, LV longitudinal deformation and LV torsion remain impaired. We suggest including a detailed study of the diastolic function and cardiac mechanics in the clinical follow-up of these patients to identify the subgroup of patients at higher risk.

A Rare Case of Anomalous Left Coronary Artery From the Pulmonary Artery (Bland-White-Garland Syndrome) in a 68-Year-Old Woman.

Anomalous left coronary artery from the pulmonary artery (ALCAPA), or Bland-White-Garland syndrome, is a rare congenital coronary anomaly that results in altered myocardial perfusion and a left to right shunt. It occurs in 1:300000 live births and represents 0.24% to 0.46% of all congenital cardiac diseases. Despite its rarity, it is one of the most common causes of ischemia and infarction in children. Ninety percent of these patients will die within the first year of life if untreated and diagnosing this abnormality in adulthood is extremely rare. Of those patients who survive to adulthood, the average age of sudden cardiac death is 35 years. The initial symptoms of the adult presentation vary widely from progressive dyspnea to sudden cardiac death; therefore, immediate surgical correction is highly recommended upon diagnosis. Understanding the pathophysiology and nature of collateral coronary flow in this congenital anomaly is paramount to the safe anesthetic management of adults with ALCAPA. Here we describe the intraoperative management and echocardiographic findings in a 68-year-old with with recently diagnosed ALCAPA undergoing surgical repair.