ABC | Volume 114, Nº4, Suplement, April 2020

Clinicoradiological Correlation Case 2/2020 – Anomalous Origin of the Left Coronary Artery from the Pulmonary Trunk, Under Natural Evolution in a 75-Year-old Asymptomatic Woman Edmar Atik , Oliver Kligerman, Luiz Kajita Instituto do Coração do Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo, São Paulo, SP – Brazil Keywords Congenital Heart Disease; BlandWhite-Garland Syndrome; Coronary Vessel Anomalies/diagnostic imaging; Ischemia. Clinical data Patient evolves without symptoms in her daily routine as a domestic worker. She denies any symptoms of palpitations, precordial pain or fatigue. When she was aged 62, a cardiac catheterization revealed the diagnosis of anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA, or Bland-White-Garland syndrome), after an altered routine exercise stress test. Since then, the patient reports her well-being, although she is aware of the existence of this anomaly. She is being treated with rosuvastatin, levothyroxine and vitamin D. Physical examination: good overall status, eupneic, acyanotic, normal pulses in the four limbs. Weight: 49.8 Kg, Height: 143 cm, BP:120 x 80 mmHg, HR: 74 bpm. Precordium: Patient presented ictus cordis impulsive and displaced to the left of the midclavicular line, without systolic impulses at left sternal border. Hyperphonetic heart sound, with a split S2. Mild continuous murmur, +/++/4, more intense in the suprasternal notch and in the 1st and 2nd left intercostal spaces. Non-palpable liver and clean lungs. Complementary examinations Electrocardiography (ECG): Junctional rhythm, with a flat P wave in the frontal plane and left precordial leads. Negative T waves in leads I, L, and of low amplitude in leads V4-V6, suggestive of anterolateral ischemia. Signs of overload of left cavities with a biphasic P wave in lead V1 and a Sokolof index equal to 37mm. QRS 102 ms (AQRS= 0º, AT= +110º) (Figure 1). Chest radiography: Slightly enlarged cardiac area, with elongated left ventricular arch (CTI=0.68). Clearly increased pulmonary vascular network (Figure 1). Echocardiography : Normal atrioventricular and ventriculoarterial connections. Significantly increased left atrium size (52 mm; LAVI = 125 ml/m 2 ). The other cavities (RV= 20, LV= 56, RA= 31), as well as the cardiac valves, were normal. There was no myocardial hypertrophy, with septum and posterior wall = 8 mm. The pulmonary artery systolic pressure was 82 mmHg as estimated by Doppler echocardiography. Biventricular function was normal and left ventricular ejection fraction was 60% (Figure 1). Cinecoronariography and cardiac catheterization: Large and extremely tortuous right coronary (RC) artery without obstruction. There was retrograde filling of the left coronary artery, which was also quite tortuous, by exuberant collateral circulation from the RC. The LC artery trunk flowed at the beginning of the dilated pulmonary trunk, with the flow originating from the RC. Left ventriculography showed preserved myocardial contractility (Figure 2). Clinical Diagnosis: Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA), under prolonged natural evolution in asymptomatic patient until 75 years of age, but with signs of myocardial ischemia, left cavities overload and preserved myocardial function. Clinical Reasoning: There were clinical elements of diagnostic orientation of congenital heart disease, despite the absence of clear symptoms. A clear continuous murmur located at the suprasternal notch and at the uppermost spaces of the left sternal border, with an increase in pulmonary Mailing Address: Edmar Atik • Private office. Rua Dona Adma Jafet, 74, conj.73, Bela Vista. Postal Code 01308-050, São Paulo, SP – Brazil E-mail: conatik@incor.usp.br DOI: https://doi.org/10.36660/abc.2019-0486 Figure 1 – Chest x-ray highlights increased cardiac area and pulmonary vascular network. ECG shows overload of left cavities with inferolateral wall ischemia of the left ventricle. A 4-chamber view echocardiogram study highlights increased left cavities. RA: right atrium; LA: left atrium; RV: right ventricle; LV: left ventricle. 40