Pulmonary hypertension in an adolescent with end-stage-renal disease—a diagnostic challenge: Answers

Excerpt

The initial hypothesis was that the heart failure was related to left ventricular (LV) diastolic dysfunction secondary to systemic thrombotic microangiopathy (TMA) and end-stage renal disease (ESRD). This is consistent with the echocardiographic findings showing dilated LV with a normal systolic function. Although LV diastolic dysfunction is associated with elevated pulmonary pressure, the results of the catheterization showed that, in our patient, pulmonary hypertension (PH) was related to a pulmonary overflow due to a massive arteriovenous left-to-right shunt at the femoral level as demonstrated by the abnormally high oxygen saturation found in the inferior vena cava. Of note, the last Doppler assessing the fistula blood flow performed in 2008 was already showing high blood flow of 2 L/min. …