Pulmonary Vasoreactivity and Phenotypes in Pulmonary Arterial Hypertension Associated to Connective Tissue Diseases.

BACKGROUND: Pulmonary arterial hypertension (PAH) is a fatal complication in patients with connective tissue disease (CTD). OBJECTIVE: The objective of the study was to study the prognostic value of the acute pulmonary vasoreactivity test with inhaled iloprost and its association with clinical deterioration in a tertiary care academic medical center. METHODS: We conducted a prospective study of patients with CTD and the diagnosis of PAH established by right heart catheterization. Patients were classified into classic responders, partial responders, and non-responders. The association of the pulmonary response and clinical deterioration was analyzed. RESULTS: We enrolled 25 patients (mean age of 47 ± 13.4 years); 88% were female. The most frequent rheumatologic diagnosis was systemic lupus erythematosus, in 16 (64%) patients. Seventy-two percent of patients were classified as non-responders, and 28% were partial responders. Patients with a partial response had lower right atrial pressure values (5.1 ± 3.1 vs. 8.5 ± 3.2, p = 0.01) and greater systolic pulmonary arterial pressure (87.6 ± 8.1 vs. 72.4 ± 16.2, p = 0.02), compared with non-responders. Non-responders had a tendency for a shorter time to clinical deterioration than partial responders (17.8 vs. 41.1 months, p = 0.052). CONCLUSIONS: Patients with a partial response to the acute pulmonary vasodilator test with inhaled iloprost had a longer clinical deterioration-free period than non-responders.

A 39-Year-Old Man With an Arteriovenous Malformation With New Dyspnea and Lower Limb Edema.

CASE PRESENTATION: A 39-year-old man with a history of arteriovenous malformation in the upper right limb that was complicated with vascular-type ulcers and repeated soft tissue infection and who had needed a supracondylar amputation of the limb when he was 27 years old presented a new soft tissue infection that manifested with fever, chills, increase in the diameter of the stump with local skin erythema, and painful necrotic ulcers. The patient reported mild dyspnea for 3 months (World Health Organization functional class II/IV) that had worsened during the last week (World Health Organization functional class III/IV) with chest tightness and bilateral lower limb edema.

A 50-Year-Old Woman With Uterine Myomatosis, Rapidly Progressive Dyspnea, and Lower Extremity Edema.

CASE PRESENTATION: A 50-year-old woman with morbid obesity (BMI, 49 kg/m2) was admitted to the ED due to shortness of breath triggered by mild to moderate efforts over the previous 3 weeks that rapidly progressed to dyspnea at rest and became associated with oppressive chest pain and edema of the lower extremities. Four months prior to admission, she had been diagnosed with a uterine mass (18 × 21 cm2) suggestive of a leiomyoma, manifesting with abnormal vaginal bleeding and microcytic hypochromic anemia (Fig 1).

A 44-Year-Old Woman With Sudden Breathlessness, Tightness in Chest, and Hypotension After Extubation in the Early Postoperative Period After Liver Transplantation.

CASE PRESENTATION: A 44-year-old woman with Child-Pugh class C cirrhosis due to primary biliary cirrhosis and mild portopulmonary syndrome received a liver transplant. Her basal catheterization showed a mean pulmonary arterial pressure (mPAP) of 28 mm Hg, pulmonary artery occlusion pressure (PAOP) of 8 mm Hg, pulmonary vascular resistance (PVR) of 307 dynes.s.cm-5, and a cardiac output of 5.2 L/min. The echocardiogram did not reveal right ventricular dilatation (mid-diameter of 34 mm). In surgery, hemodynamic assessment showed an mPAP of 25 mm Hg, PAOP of 6 mm Hg, PVR of 262 dynes.s.cm-5 and cardiac output of 5.8 L/min. During the anhepatic period, the patient required norepinephrine (0.4 µg/kg/min) but had no complications during reperfusion; throughout surgery, her mPAP was never > 30 mm Hg. At the end of surgery, the brain natriuretic peptide level was 66 pg/mL (< 100 pg/mL). One day following transplantation, the patient remained hemodynamically stable and was therefore weaned from mechanical ventilation. However, 6 h following extubation, she reported breathlessness and tightness in chest, and developed sudden arterial hypotension, oxygen desaturation, and oliguria.