Síndrome de ALCAPA em uma Mulher Jovem / ALCAPA Syndrome in a Young Woman

Mulher de 18 anos com histórico de síncope, angina e palpitações há um ano. Uma indicação crucial era artéria coronária direita dilatada na ecocardiografia transtorácica. Os achados da tomografia computadorizada resultaram no diagnóstico da origem anômala da artéria coronariana esquerda proveniente da síndrome da artéria pulmonar.(AU)

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 origin of the left coronary artery from the pulmonary artery in a patient of advanced age: a case report and analysis.

A 61-year-old Chinese man presented with a nearly 30-year history of an anomalous origin of the left coronary artery. He had been diagnosed with an anomalous origin of the left coronary artery in 1989. He then underwent regular echocardiographic examinations and it was found that his heart was gradually enlarging. After a >20-year asymptomatic period, he developed recurrent chest discomfort and palpitation. Coronary computed tomography angiography suggested that the left coronary artery anomaly originated from the pulmonary artery; additionally, the right coronary artery was tortuous and thickened. Coronary angiography showed that the right coronary artery was huge and buckling. The patient underwent corrective surgery of the anomalous origin of the left coronary artery from the pulmonary artery, aortic valve mechanical valve replacement, mitral valve plasty, and tricuspid valve plasty in Fuwai Hospital (National Center of Cardiovascular Disease of China), and the anatomic results of the surgery were good.

Two congenital coronary abnormalities affecting heart function: anomalous origin of the left coronary artery from the pulmonary artery and congenital left main coronary artery atresia.

BACKGROUND: The anomalous origin of the left coronary artery (LCA) from the pulmonary artery (ALCAPA) and congenital left main coronary artery atresia (CLMCA-A) are two kinds of very rare coronary heart diseases which affect heart function profoundly. This study aimed to retrospectively illustrate the clinical features and therapy experience of ALCAPA and CLMCA-A patients. METHODS: From April 1984 to July 2012, in Beijing Anzhen Hospital, 23 patients were diagnosed with ALCAPA and 4 patients with CLMCA-A. We summarized the clinical data of the 27 cases and retrospectively analyzed the clinical manifestation, diagnosis, and treatments of these two kinds of congenital coronary abnormalities. RESULTS: The 23 patients (13 males and 10 females, aged ranging from 2.5 months to 65 years) identified with ALCAPA were classified into infantile type (age of onset younger than 12 months, 16 cases) and adult type (age of onset older than 12 months, 7 cases). Four patients were diagnosed with CLMCA-A (three males and one female, aged ranging from 3 months to 2 years). The main clinical manifestations of infantile-type ALCAPA and CLMCA-A include repeated respiratory tract infection, heart failure, dyspnea, feeding intolerance, diaphoresis, and failure to thrive. And these two congenital coronary abnormalities might be misdiagnosed as endocardial fibroelastosis, dilated cardiomyopathy, and acute myocardial infarction. As for the adult-type ALCAPA, cardiac murmurs and discomfort of the precordial area are the most common presentations and might be misdiagnosed as coronary heart disease, myocarditis, or patent ductus arteriosus. In ECG examination: Infantile-type ALCAPA and CLMCA-A showed abnormal Q waves with T wave inversion in leads I, avL, and V4-V6, especially in lead avL. However, ECG of adult-type ALCAPA lacked distinct features. In chest radiography: pulmonary congestion and cardiomegaly were the most common findings in infantile-type ALCAPA and CLMCA-A, while pulmonary artery segment dilation was more common in adult type. In echocardiography, the common features of infantile-type ALCAPA and CLMCA-A included left ventricular enlargement, left ventricular systolic function normal or mildly reduced in CLMCA-A or significantly reduced in ALCAPA, and moderate to large mitral valve. It was performed in 9 of 23 cases of ALCAPA and showed the origin of the dilated right coronary artery (RCA) from the right sinus of the aortic root and absence of LCA origin in angiography. After opacification of RCA, reverse flow in the LCA and pulmonary artery was visualized through coronary artery collateral circulation. Angio was performed in three of the four cases of CLMCA-A and showed left main coronary artery was a blind end, with diameter of only 1.1-2.0 mm. Treatment and prognosis: 21 patients with ALCAPA had cardiac surgery and 6 of them died postoperatively. Fifteen postoperative patients survived without overt symptoms within the follow-up period of 6-166 months (median 17 months). As for treatment of CLMCA-A, four patients took digoxin and diuretics without undergoing cardiac surgery. Their clinical symptoms improved during the close follow-ups. CONCLUSIONS: ALCAPA and CLMCA-A are two rare coronary artery abnormalities that affect cardiac function in infants and children. In younger patients with cardiomegaly and heart dysfunction these two congenital coronary diseases should be noticed.

[The medicolegal relevance of benign and malignant coronary artery anomalies]. / Uber die Relevanz von benignen und malignen Koronaranomalien in der Rechtsmedizin.

Isolated coronary anomalies are rare abnormalities that can be divided into benign and malignant forms. Malignant coronary artery abnormalities generally present already during infancy or early childhood by causing symptoms or sudden death. Benign abnormalities often remain asymptomatic because they are hemodynamically irrelevant. Among the 12,985 autopsies performed at the Institute of Legal Medicine at the University of Frankfurt (Germany) between 1995 and 2013, there were eight cases (0.062%) with isolated coronary artery abnormalities. Five of these cases (0.039%) could be included in our retrospective study. These involved one deceased female and four deceased male subjects, aged between 2 and 57 years. In three of these cases (aged 6, 9, and 11 years old), the coronary anomaly was classified as malignant and was recorded as the cause of death. Benign isolated coronary anomalies are often incidental findings during autopsies. However, in one of the cases in our study, coronary sclerosis in precisely the anomalous vessel was found to be the origin of a fatal myocardial infarction. Malignant isolated coronary artery anomalies attain medicolegal significance when they remain undetected despite advanced early detection protocols and cause sudden death in childhood.

Cardiovascular magnetic resonance assessment of ventricular function and myocardial scarring before and early after repair of anomalous left coronary artery from the pulmonary artery.

BACKGROUND: In patients with anomalous left coronary artery from the pulmonary artery (ALCAPA) left ventricular (LV) dilatation and dysfunction evolves due to diminished myocardial perfusion caused by coronary steal phenomenon. Using late gadolinium enhanced cardiovascular magnetic resonance (LGE-CMR) imaging, myocardial scarring has been shown in ALCAPA patients late after repair, however the incidence of scarring before surgery and its impact on postoperative course after surgical repair remained unknown. METHODS: 8 ALCAPA-patients (mean age 10.0 ± 5.8 months) underwent CMR before and early after (mean 4.9 ± 2.5 months) coronary reimplantation procedures. CMR included functional analysis and LGE for detection of myocardial scars. RESULTS: LV dilatation (mean LVEDVI 171 ± 94 ml/m2) and dysfunction (mean LV-EF 22 ± 10 %) was present in all patients and improved significantly after surgery (mean LVEDV 68 ± 42 ml/m2, p=0.02; mean LV-EF 58 ± 19 %, p<0.001). Preoperative CMR revealed myocardial scarring in 2 of the 8 patients and did not predict postoperative course. At follow-up CMR, one LGE-positive patient showed delayed recovery of LV function while myocardial scarring was still present in both patients. In two patients new-onset transmural scarring was found, although functional recovery after operation was sufficient. One of them showed a stenosis of the left coronary artery and required resurgery. CONCLUSIONS: Despite diminished myocardial perfusion and severely compromised LV function, myocardial scarring was preoperatively only infrequently present. Improvement of myocardial function was independent of new-onset scarring while the impact of preoperative scarring still needs to be defined.

Multimodality imaging in bland-White-Garland syndrome in an adult with a left dominant coronary artery system.

Bland-White-Garland syndrome, also known as anomalous origin of the left coronary artery from the pulmonary artery, is a rare congenital disorder affecting around one in 300,000 live births. The majority of these present within the first year of life with 90% mortality rate if untreated and consequently is an extremely rare presentation in the adult. We present the first published case with a left dominant system in an adult presenting in their late 20s, illustrated by multimodality imaging.

Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA), a forgotten congenital cause of sudden death in the adult.

Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a very rare often fatal congenital coronary artery anomaly that occurs in approximately only 1 of 300,000 live births. Many cardiologists and pathologists may be unaware that cardiac ischemia and sudden death can also occur due to ALCAPA in adults in whom it is even rarer and thus may be missed at autopsy. The adult form may range clinically from asymptomatic to sudden cardiac death. We present four cases of adult ALCAPA that illustrate the variable presentations and survival. Two had died at rest and were previously asymptomatic. The other two died during exertion with history of recent chest pains in one and previous myocardial infarction in childhood in the other. This last case remained asymptomatic until death. These four cases highlight that surgery should be undertaken in all cases, even in those who are asymptomatic as newer imaging makes the diagnosis in life more frequent. Age is not important as cases vary widely in age from 22 to 68 in our small group. Furthermore, the most common site of left coronary artery origin is the left pulmonary sinus but our autopsy study shows that the origin can be variable extending above the pulmonary valve sinus to the main pulmonary artery.