Pulmonary Artery Syndrome Research Articles

Accidental finding of ALCAPA in a child with severe mitral regurgitation: A case study.

A six-year-old child who had undergone cardiac surgery five years ago presented to us with severe mitral valve regurgitation. During her current surgery, the coronary artery was injured during the attempt to release extensive epicardial adhesion, resulting in very poor contractility that prompted a delay in the intended valve repair. The injured coronary vessels were successfully repaired, yet low cardiac output syndrome persisted during perioperative care, necessitating further investigation of the coronary problem. It was later discovered that the patient had anomalous left coronary artery from pulmonary artery (ALCAPA) syndrome. The presence of severe mitral regurgitation, pulmonary hypertension, and anatomical factors may have contributed to the delayed presentation and diagnosis in this case. The severity of mitral regurgitation and the most likely underlying mechanism indicates a low possibility of recovery following coronary repair alone, warranting the need for concomitant mitral surgery. Coronary and mitral repair were performed in this patient, resulting in a favorable outcome. The management of ALCAPA presents unique challenges, especially in cases with delayed diagnosis. Proper diagnosis and tailored surgical approaches are crucial for achieving favorable outcomes in patients with ALCAPA.

Anomalous Left Coronary Artery from the Pulmonary Artery (ALCAPA) Syndrome: Pathophysiology, Clinical Manifestations, and Management Strategies

Anomalous Left Coronary Artery from the Pulmonary Artery (ALCAPA) syndrome is a rare congenital cardiac anomaly characterized by the abnormal origin of the left coronary artery from the pulmonary artery instead of the aortic root. This anomaly results in compromised myocardial perfusion, particularly under conditions of increased oxygen demand, leading to myocardial ischemia, left ventricular dysfunction, and potentially severe complications such as mitral regurgitation, heart failure, and sudden cardiac death. The pathophysiology of ALCAPA involves a complex interplay of coronary artery steal phenomenon, collateral vessel development, and ventricular remodeling. Early diagnosis is critical, often achieved through echocardiography, cardiac MRI, or coronary angiography, which helps in planning surgical intervention. The mainstay of treatment is surgical correction to re-establish a dual coronary artery system, with techniques including direct reimplantation of the anomalous coronary artery into the aorta or the creation of an intrapulmonary tunnel. This article provides a comprehensive review of the current understanding of ALCAPA syndrome, emphasizing its pathophysiology, clinical presentation, diagnostic modalities, and surgical management, along with outcomes and prognostic factors.

Clinical manifestations and computed tomography angiography features of anomalous left coronary artery from the pulmonary artery syndrome.

Anomalous left coronary artery from the pulmonary artery (ALCAPA) syndrome is a rare congenital coronary artery malformation with a high rate of clinical missed diagnosis and misdiagnosis. At present, there is a lack of reports on the clinical manifestations of ALCAPA and the imaging features of coronary computed tomography angiography (CTA). This study aims to summarize the clinical characteristics and coronary CTA imaging features of ALCAPA to improve the clinical diagnosis and treatment. The clinical data of 24 ALCAPA syndrome patients were retrospectively collected, analyzed and summarized from January 1, 2013 to February 1, 2023 in the Second Xiangya Hospital of Central South University. According to the left and right coronary collateral circulation, the patients were classified into infantile type (7 cases) and adult type (17 cases). The differences of clinical manifestations and CTA imaging features were compared between the 2 types of patients. Of the 24 patients, a male-to-female ratio was at 1꞉5, and the median age of onset was 3.22 months for the infant type and 22.0 years for the adult type. The infantile type showed symptoms of left heart insufficiency at an early stage, while the adult type had a variety of clinical manifestations in 7 patients being asymptomatic and presented with a cardiac murmur on physical examination, 6 with symptoms of chronic myocardial ischemia, and 2 with malignant arrhythmias. CTA showed that 11 patients' the left coronary artery originated from the left posterior sinus of the pulmonary artery. All 7 infantile type patients had an enlarged left heart, left ventricular hypertrophy, reduced left heart function, lack of collateral circulation between the left and right coronary arteries, and normal coronary artery diameter. All 17 adult type patients showed dilated and tortuous coronary arteries with rich collateral circulation, and 7 adult type patients had preserved left heart function. The clinical manifestations and CTA imaging features of patients with the 2 types of ALCAPA are different, while CTA performance is characteristic and can be used as a means of definitive diagnosis, staging, surgical evaluation, and postoperative follow-up of ALCAPA syndrome.

ДИАГНОСТИЧЕСКИЕ ВОЗМОЖНОСТИ В ОЦЕНКЕ ОТДАЛЕННЫХ РЕЗУЛЬТАТОВ ХИРУРГИЧЕСКОГО ЛЕЧЕНИЯ АНОМАЛЬНОГО ОТХОЖДЕНИЯ ЛЕВОЙ КОРОНАРНОЙ АРТЕРИИ ОТ ЛЕГОЧНОЙ АРТЕРИИ

Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) syndrome is a rare congenital coronary artery anomaly and a complex congenital heart defect (CHD) condition. According to the bibliographical sources a good result is noted with the restoration of the heart size and its functional state after the CHD correction. Nevertheless, after a radical correction of the defect, a number of problems may remain, such as: the occurrence of malignant arrhythmias, sudden cardiac death, decompensated heart failure (HF), coronary artery stenosis that require medical or repeated surgical treatment. The detection of even minor deviations in the functional state of the myocardium in the population of patients with ALCAPA is of great clinical importance, and this category of patients requires the regular and dynamic follow-up. The evolution of diagnostic research methods, such as biomarkers of heart failure, echocardiography with tissue myocardial Doppler and speakle tracking, magnetic resonance imaging, single photon emission computed tomography, positron emission tomography and stress testing, provide opportunity to assess the functional and anatomical recovery of the left ventricular myocardium in detail and to identify the signs of latent HF in the late postoperative period. The purpose of the research was to provide an up-to-date review of the data on the diagnostic and prognostic significance of laboratory and instrumental research methods in assessing the long-term results of surgical treatment of the anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) syndrome.

Anomalous left coronary artery from the pulmonary artery syndrome: When everything falls into place

Anomalous left coronary artery from the pulmonary artery syndrome: When everything falls into place

A Rare Case of ALCAPA in Adult Asymptomatic Patient and Unusual Collateral Circulation: A Case Report

Anomalous Origin of the Left Coronary Artery from Pulmonary Artery (ALCAPA) syndrome is a rare congenital anomaly where the left main coronary artery arises from the pulmonary artery (PA) and the right coronary artery (RCA) originates normally from the aorta. The rapid decrease in pulmonary vascular resistance (PVR) soon after birth reverses the blood flow to the PA resulting in a left to right shunt and severe left ventricle (LV) ischemia. Left untreated, this congenital anomaly is associated with premature death. Rarely, some patients can survive to adult age due to development of collateral circulation usually from an ecstatic RCA. Most adult patients with ALCAPA die at mean age of 35 years. Very few cases were reported in the literature to survive until late 40s or 50s. We present the case of ALCAPA in an asymptomatic 54-year-old woman who found to have unusual collateral circulation justifying the absence of significant symptoms. Our patient underwent surgical correction with good results. We also review cases of ALCAPA that has been reported in the literature and we summarize treatment strategies. Learning objective: recognition of ALCAPA as one of coronary anomalies in adults even in asymptomatic individuals and recognition of the best diagnostic modality and the optimal management according to available literature.

Anomalous origin of left coronary artery from the pulmonary artery syndrome of an 18-year-old Palestinian male: case report

Anomalous origin of left coronary artery from the pulmonary artery syndrome of an 18-year-old Palestinian male: case report

Anomalous origin of the left coronary artery from the pulmonary artery syndrome in a young adult

Anomalous origin of the left coronary artery from the pulmonary artery syndrome in a young adult

Síndrome de ALCAPA em uma Mulher Jovem

An 18-year-old woman presented with a one-year history of syncope, angina, and palpitations. The critical clue was a dilated right coronary artery on transthoracic echocardiography. Computed tomography findings resulted in the diagnosis of anomalous origin of the left coronary artery from the pulmonary artery syndrome.

Erratum: Anomalous Left Coronary Artery from the Pulmonary Artery Syndrome: A Case Report in a 49-Year-Old Woman

Erratum: Anomalous Left Coronary Artery from the Pulmonary Artery Syndrome: A Case Report in a 49-Year-Old Woman

Anomalous Left Coronary Artery from the Pulmonary Artery Syndrome: A Case Report in a 49-Year-Old Woman

Anomalous Left Coronary Artery from the Pulmonary Artery Syndrome: A Case Report in a 49-Year-Old Woman

Adult-Type Anomalous Origin of the Left Coronary Artery from the Pulmonary Artery Syndrome

Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare cardiac malformation accounting for 0.25% of congenital heart diseases with reported incidence of 1 in 300,000 live births. It frequently occurs as an isolated defect and is associated with early infant mortality and sudden death in adults. Surgical repair is the treatment of choice and should be done soon after diagnosis. We report the case of a 38-year-old female, presenting as cardiac arrest and being finally diagnosed as adult ALCAPA. Successful surgical repair was performed using the trap door technique. The patient had an uneventful recovery.

P1338 Anomalous left coronary artery from pulmonary artery syndrome diagnosed in adulthood: case of a mother of five

Abstract Funding Acknowledgements Project no. NVKP_16-1-2016-0017 has been implemented with the support provided from the National Research, Development and Innovation Fund of Hungary Introduction Bland-White-Garland syndrome or ALCAPA (anomalous left coronary artery from pulmonary artery) syndrome is a rare congenital disorder with a prevalence of 1: 300,000. This condition is one the most common causes of infant myocardial infarction. It can ultimately lead to heart failure before the age of one, however when pronounced left and right coronary artery collateralization is present, patients may remain asymptomatic until older age. Due to the development and availability of non-invasive imaging techniques, these patients diagnosed more frequently in recent years. Case description: We present a 53-year-old patient with treated hypertension and five uncomplicated pregnancies. At the age of 40, the patient was referred to coronary angiography due to her dyspnea on exertion. The examination showed that the left main coronary artery (LMCA) originates from the pulmonary artery 7 mm above the pulmonary valve and the right coronary artery is significantly dilated. Based on the mild complaints and good left ventricle systolic function, conservative therapy was recommended by the cardiac surgery team. Since 2018 the patient has had gradually worsening complaints despite of the therapy adjusted for chronic heart failure. Q-waves were depicted on the 12-lead ECG in aVL and V1-V6 leads. Echocardiography showed dilated left ventricle (LV), LV hypertrophy, diffuse hypokinesia, decreased LV systolic function and diastolic dysfunction. At our clinic, we performed cardiac magnetic resonance imaging (CMR) which showed moderately reduced LV ejection fraction, diffuse LV hypokinesia, increased LV volume, septal LV hypertrophy. We visualized the retrograde flow on the LMCA, the shunt volume at the anomalous artery was 1,27 L/min. The CMR also showed irreversible damage of the subendocardial region of the myocardium via late gadolinium enhancement in the area supplied by the LMCA. Since we planned to reevaluate the surgical possibilities, the collateral network with tortuous and dilated left and right coronary arteries were visualized by coronary CT angiography. The surgical team at our institute decided to perform myocardial revascularization and restoration of dual coronary artery supply. Discussion ALCAPA is usually diagnosed in infancy however, in about 10-15% of cases the disease is only recognized in adulthood. The long-term morphological and functional characteristics of the syndrome may include reversible/irreversible ischemic damage, systolic and diastolic dysfunction, mitral regurgitation, significant collateral circulation, left-to-right shunt, dilated coronary arteries. Surgical myocardial revascularization and restoration of dual coronary artery supply is recommended even in adult patients. Abstract P1338 Figure. CTA reconstruction and LGE CMR

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

BackgroundAnomalous 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.AimDetermination of the incidence of incipient myocardial dysfunction in ALCAPA patients in the long-term observation using tissue Doppler and speckle tracking echocardiography.Methods and resultsEighteen 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).ConclusionsDespite 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.

DYSPNEA OF A RARE ETIOLOGY: THE ALCAPA SYNDROME

The Anomalous Origin of the Left Coronary Artery from the Pulmonary Artery(ALCAPA) Syndrome is a rare congenital coronary anomaly that is associated with 90% infant mortality. Survivors have an increased risk of sudden cardiac death during the 3rd to 4th decades of life. A 43-year-old female

Anomalous origin of the left coronary artery from the pulmonary artery-adult type with heart failure and ventricular arrhythmia presentation

Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) syndrome is a rare congenital anomaly occurring in 1 of 300,000 births. As it has high mortality rate during the 1st year of life, adult type is very rare. Arrhythmias, heart failure, myocardial infarction, and sudden cardiac death are common presentations of ALCAPA syndrome in adults. Here, we report a 28-year-old male with malignant ventricular arrhythmia and sudden cardiac death. ALCAPA syndrome was diagnosed by coronary angiography and echocardiography; finally, cardiac surgery was performed with a good result.

An unusual presentation of anomalous left coronary artery from the pulmonary artery (ALCAPA) syndrome in a 70-year-old man: a case report

BackgroundWe present a rare case of anomalous origin of the left coronary artery from the pulmonary artery syndrome in an elderly man, and we describe coronary computed tomographic angiographic imaging findings to improve diagnostic confidence for the evaluation of this uncommon coronary artery anomaly.Case presentationA 70-year-old Caucasian man came to our hospital with slight limitation of physical activity (New York Heart Association class II). He was asymptomatic for angina, syncope, and palpitations. Cardiac magnetic resonance imaging was performed after echocardiography because a hypertrophic cardiomyopathy was suspected; a plausible coronary artery anomaly was demonstrated as collateral evidence. Subsequently, coronary computed tomographic angiography showed the anomalous origin of left coronary artery from the pulmonary artery; the coronary vessels appeared markedly dilated and tortuous. Dilated intercoronary vessels along the epicardial surface of the heart and dilated bronchial arteries, corresponding to collateral pathways, were observed. Left ventricular hypertrophy, delayed subendocardial enhancement, and mitral insufficiency were better evaluated on magnetic resonance images. Invasive coronary angiography confirmed the main findings. Given the patient’s age and clinical performance, surveillance with medical management was considered appropriate, and surgical repair was avoided.ConclusionsConfidence with the anatomic pattern and clinical significance of this anomalous condition is necessary to improve cardiac imaging evaluation ability. In our patient, coronary computed tomographic angiography proved to be a reliable imaging approach, superior to invasive coronary angiography in terms of diagnostic performance and patient safety.

A RARE CAUSE OF PERIPARTUM CARDIOMYOPATHY

Anomalous origin of the left coronary artery from pulmonary artery (ALCAPA) syndrome is a rare disease that accounts for 0.2-0.5% of all congenital heart defects. If untreated, 90% of patients die within the 1st year of life. A 30-year-old woman presented with chest pain and dyspnea on exertion

Connecting the Dots: VF Arrest Secondary to Anomalous Origin of the Left Coronary Artery from the Pulmonary Artery Syndrome

A 16-year-old male water polo player with no significant past history, was admitted to the current institution in cardiogenic shock following an out-of-hospital ventricular fibrillation arrest whilst swimming. The patient had return of spontaneous circulation following timely defibrillation for monomorphic left ventricular (LV) tachycardia. Transthoracic echocardiography (TTE) demonstrated a severely dilated LV, with severe global systolic dysfunction and an estimated LV ejection fraction (LVEF) of 30%. Troponin I assay was 5,760 ng/L. Intravenous methylprednisolone was commenced empirically for presumed myocarditis. Endomyocardial biopsy demonstrated mild non-specific cardiomyopathic changes and an increased number of blood vessels suggestive of a vascular malformation. Left ventricular systolic function improved, with repeat TTE a week after admission showing an LVEF of 50%. Cardiac magnetic resonance imaging showed a dilated LV with increased wall thickness and vascularity, primarily septal, and evidence of an old distal anterior myocardial infarct with wall thinning and transmural late gadolinium enhancement. Coronary angiography showed a large-calibre, ectatic right coronary artery, with retrograde filling of a highly abnormal left-sided system that could not be engaged. Computed tomography coronary angiogram was subsequently performed, demonstrating an anomalous left main coronary artery arising from the main pulmonary artery. There was a large dominant right coronary artery with multiple collaterals. This was a rare case of anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA syndrome), in which survival to adulthood was due to extensive collateralisation from the right coronary system. Silent myocardial ischaemia and infarct is not uncommon, as was seen in this patient. The patient underwent successful reimplantation of the left coronary artery to the aorta at a quaternary paediatric cardiothoracic referral centre.

Missed Diagnosis and Treatment Dilemma: Large Patent Ductus Arteriosus Combined with Anomalous Origin of the Left Coronary Artery From the Pulmonary Artery

: An anomaly of the left coronary artery, originating from the pulmonary trunk, is called anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) syndrome. This syndrome is an infrequent congenital anomaly, mostly seen in children. Usually, because of the anomaly, myocardial perfusion failure occurs. This anomaly presents even less frequently in adults. The symptoms of ALCAPA develop in the early ages. Congestive heart failure is an important result of ALCAPA, as well as mitral insufficiency and left ventricular (LV) dysfunction. If there is a patent ductus arteriosus (PDA) or ventricular septal defect (VSD), high pulmonary artery pressures decreases and the coronary anomaly and perfusion defect can be hidden. Here we present a case of a large PDA combined with ALCAPA, as seen in one of the oldest patients ever reported. Recognizing PDA in patients with ALCAPA is very important, since closure of the PDA may cause catastrophic conditions. ALCAPA combined with PDA is very rare and has been reported mostly in infants. To the best of our knowledge, there is no such case diagnosed in adulthood.