A 33-year-old female with carcinoid heart disease and a 27-mm Medtronic-Hall tricuspid valve prosthesis presented with dyspnea 2 weeks after abdominal surgery. Initial transthoracic echocardiography (TTE) demonstrated a 6 -mm Hg diastolic gradient and appropriate opening and closing of the prosthetic valve (A; Online Videos 1A and 1B). Within hours, the patient required intubation for hypoxemia. Invasive hemodynamic assessment revealed a central venous pressure (CVP) of 30 mm Hg with a biphasic dip and rise every 5 to 7 cardiac cycles (B). Arterial blood pressure varied inversely with the CVP. The suspected diagnosis of an intra-atrial shunt causing hypoxemia (C; Online Video 2) secondary to intermittent obstruction of the prosthetic valve (D; Online Video 3) was confirmed by using 2-dimensional transesophageal echocardiography and pulsed-wave Doppler imaging. Valve opening corresponded to an accentuated x descent, v wave, and y descent in the CVP tracing. A thrombus was seen in the right atrium (RA) (C; Online Video 2), and thrombolytic therapy resolved the valve dysfunction, CVP overload, and hypoxemia. LA ϭ left atrium; RV ϭ right ventricle; SVC ϭ superior vena cava.

A 27-year-old G3P1011 presented at 34 weeks gestation with dyspnoea, which she also reported in her prior pregnancy. Her dyspnoea was accompanied by exertional hypoxemia. After excluding other etiologies of hypoxia such as cardiac anomalies and pulmonary embolism, arterio-venous shunting became the working diagnosis. She was treated successfully with ambulatory oxygen therapy and her symptoms resolved spontaneously after delivery with a good maternal and fetal outcome. Although we were unable to confirm a diagnosis of arteriovenous malformation of hereditary hemorrhagic telangiectasia, we remain suspicious that this is the underlying cause of her symptoms. Review of hereditary hemorrhagic telangiectasia identifies concerns regarding screening and obstetric management. Hereditary hemorrhagic telangiectasia is a rare genetic disease that may present as transient exertional hypoxemia in the third trimester of pregnancy. Pregnancy with arteriovenous shunting can be managed with symptomatic treatment along and careful attention to existing obstetric and anesthetic guidelines.

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