An 18-year-old male with dyspnea and pulmonary arterial hypertension

Abstract

We report the case of an 18-year-old white male complaining of sporadic episodes of dyspnea. The patient reported that one week earlier, edema in the lower limbs appeared and progressively extended to the abdomen and upper limbs. The patient also reported that on the day before he had experienced dyspnea. He then sought medical assistance and was hospitalized. The patient denied any pathologic antecedent and the use of tobacco, alcohol, and drugs.On physical examination, the patient was cyanotic (++/4). His respiration rate was 27 breaths per minute, his heart rate was 98bpm, and his blood pressure was 110/ 60mmHg. His central venous pressure remained between 27 and 29cm of water. His pulmonary examination was considered within the normal range. On cardiac auscultation, his heart rhythm was regular with increased intensity of the second cardiac sound in the pulmonary area and a systolic murmur of +/4 in the tricuspid area. His abdomen examination revealed mild and painless hepatomegaly. Edema was observed in the lower and upper limbs and in the abdominal wall, as well.A chest X-ray in the anteroposterior projection showed moderate enlargement of the cardiac silhouette due to the right atrium and bulging of the left pulmonary trunk segment ( fig. 1).The electrocardiography showed sinus rhythm, heart rate of 100bpm, QRS axis of +120°, morphology of right bundle-branch block in V1 with a QRS duration of 0.08s, and a deep S wave in V5 and V6, suggesting right ventricular hypertrophy ( figs. 2A and B).The catheterization of the pulmonary artery through venous via revealed pulmonary artery pressure of 48mmHg (normal = 25mmHg) and pulmonary vascular resistance of 724 dines/s/cm 5 (normal = 225 -315 dines/s/cm 5 ). The pulmonary capillary pressure remained normal. The hemogram, assessment of the renal function, coagulation tests, and urinary sediment examination were all normal.A few hours after hospitalization, the patient suddenly experienced shalon breathing, bradycardia, total atrioventricular block, and evolved with electromechanical dissociation. The patient did not respond to the resuscitation maneuvers and died.Diagnostic hypotheses -1) Heart failure due to rheumatic valvular disease -mitral stenosis; 2) Primary pulmonary hypertension. (Medical student Marcos Bianchini Cardoso) DiscussionClinical features -Pulmonary hypertension is defined as a pressure in the pulmonary arterial system higher than 25mmHg at rest and higher than 30mmHg during exercise 1 .Primary pulmonary hypertension is a rare condition that is clinically defined after exclusion of all cardiac and pulmonary diseases that may cause elevation of the pressure in the pulmonary artery and elevation of vascular resistance. Its major symptoms are dyspnea, thoracic pain, and syncope, and, on examination, signs of different grades of cor pulmonale may be present. Among the causes of secondary pulmonary hypertension we emphasize pulmonary