ABC | Volume 115, Nº1, Suplement, July 2020

Clinicoradiological Correlation Case 6/2020 – 16-Year-Old Adolescent with Severe Pulmonary Stenosis At Valvar Level, After Correction of Truncus Arteriosus using the Barbero-Marcial Technique in the First Month of Life Edmar Ati k and Miguel Barbero-Marcial Dr Edmar Atik private clinic Keywords Heart Defects,Congenital; Heart Failure; Truncus Arteriosus/ surgery; Barbero-Marcial Procedure; Diagnostic, Imaging. Clinical Data The newborn, in heart failure with truncus arteriosus type I, underwent repair at 15 days of age, weighing 2800 g. of body weight, by the Barbero-Marcial technique. At that time, the right ventricular outflow tract was approached directly with the pulmonary trunk and a monocusp valve was placed in the pulmonary position. The evolution was adequate, with heart failure control, and he remained asymptomatic and showed normal physical development. The clinical examination ruled out residual lesions, such as pulmonary valve insufficiency. Over time, while asymptomatic, a systolic murmur was identified in the pulmonary area, of progressive intensity, together with an increasing pressure gradient in the region of the pulmonary monocusp. At 2 years of age, it was 25 mmHg; at 5 years, 34 mm Hg; at 7 years, it was 40; at 13 years it was 90 and at 16 years it was 149 mmHg. The patient did not use any specific medications. Physical Examination: good overall status, eupneic, acyanotic, normal pulses. Weight: 60 Kgs, Height: 165 cm, BP: 110/70 mm Hg, HR: 73 bpm. The aorta was nonpalpable at the suprasternal notch. In the precordium, the apical impulse was nonpalpable and there were no systolic impulses in the left sternal border (LSB). The heart sounds were hyperphonetic and a +/++/4 rough systolicmurmur was auscultated in the pulmonary area and along the LSB. Nonpalpable liver and clear lungs. Complementary Examinations Electrocardiogram showed sinus rhythm and signs of complete right bundle-branch block. AQRS =+160 o , AP and AT = 50 o C. The QRS duration was 0.13”. There were no left ventricular potentials, with rR’ morphology in V1 and RS in V6. Chest x-ray showed moderately increased cardiac area on account of the atrial and ventricular arches and normal pulmonary vascular network. Cardiomegaly was progressive since the surgical correction, with a current cardiothoracic index of 0.60 (figure 1). Echocardiogram showed a well-positioned interventricular patch and no residual shunt. The right cavities were moderately dilated and showed ventricular dysfunction. The RV also showed hypertrophy. The maximum gradient between the RV and the pulmonary trunk was 149 mmHg, with an average of 86 mmHg. The dimensions were: Ao = 32, LA = 28, RV = 34, LV = 41, septum = posterior wall = 7, LV function = 66%, RPA = 22 and LPA = 26 mm. Mild pulmonary insufficiency. Cardiac tomography showed normal-sized atria, right ventricle with medio-apical hypertrophy and RVEDV = 135.2 mL/ m 2 and RV dysfunction = 28%. The RV outflow tract showed a calcified monocusp and the planimetry of the region showed the valve opening was 0.95 cm 2 , with a diameter of 14.3 x 6.2 cm. The interventricular septum was intact, and the aorta had a normal caliber. Measures of interest: 1) Aortic root: 35.4 x 35.0mm (Z-score 3.3). 2) Ascending aorta: 27.6 x 25.2mm. 3) Proximal aortic arch: 22.1 x 20.4mm - mean: 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.20190490 Figure 1 – Chest x-ray highlights the moderate increase in the cardiac area on the account of the right cavities, with normal pulmonary vascular network. 37