Portopulmonary Hypertension: An Updated Review.

Portal hypertension may have major consequences on the pulmonary vasculature due to the complex pathophysiological interactions between the liver and lungs. Portopulmonary hypertension (PoPH), a subset of group 1 pulmonary hypertension (PH), is a serious pulmonary vascular disease secondary to portal hypertension, and is the fourth most common subtype of pulmonary arterial hypertension. It is most commonly observed in cirrhotic patients; however, patients with noncirrhotic portal hypertension can also develop it. On suspicion of PoPH, the initial evaluation is by a transthoracic echocardiogram in which, if elevated pulmonary pressures are shown, patients should undergo right heart catheterization to confirm the diagnosis. The prognosis is extremely poor in untreated patients; therefore, management includes pulmonary arterial hypertension therapies with the aim of improving pulmonary hemodynamics and moving patients to orthotopic liver transplantation (OLT). In this article, we review in detail the epidemiology, pathophysiology, process for diagnosis, and most current treatments including OLT and prognosis in patients with PoPH. In addition, we present a diagnostic algorithm that includes the current criteria to properly select patients with PoPH who are candidates for OLT.

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.

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.

Sarcoidosis: a single hospital-based study in a 24-year period.

BACKGROUND: Clinical presentation of sarcoidosis varies according to race and geographical area. We describe the clinical spectrum and outcome of sarcoidosis in Mexican patients compared with other populations. METHODS: We reviewed the medical charts of 21 patients with sarcoidosis seen at a referral hospital in 1989-2012; organ involvement was assessed using the ACCESS instrument. We compared our results with the ACCESS and Latin American studies. We used descriptive statistics and reported odd ratios with 95% CI. RESULTS AND CONCLUSION: Fifty-two percent were women; median age was 31 years; median time to diagnosis, 5.5 months. Frequency of organ involvement was: constitutional symptoms 62%, lungs 66.6%, skin 42.8%, bone marrow 23.4%, lymph node 19%, liver 19%, and eye 19%. After one year of follow-up, 47.5% of patients were asymptomatic without treatment, 38% asymptomatic on treatment, and 14.2% symptomatic on treatment. In our patients, pulmonary involvement was lower (66.6 vs. 94.9%; p = 0.001) and cutaneous (42.8 vs. 15.8%; p = 0.003) and bone marrow (23.4 vs. 4.7%; p = 0.001) were higher than in the ACCESS cohort. Data regarding Latin American populations was scarce. The clinical spectrum of sarcoidosis in our population differed from other studies, with a higher frequency of cutaneous sarcoidosis and less pulmonary involvement.

[Pulmonary hypertension associated with connective tissue diseases]. / Hipertensión arterial pulmonar por ecocardiografía en pacientes con enfermedades de tejido conectivo.

BACKGROUND: Pulmonary arterial hypertension is an important cause of complications among patients with connective tissue diseases. AIM: To describe the clinical and echocardiographic characteristics of patients with pulmonary hypertension associated with connective tissue diseases. MATERIAL AND METHODS: Retrospective, observational and descriptive study. We analyzed 35 patients with pulmonary hypertension associated with connective tissue diseases. All patients were evaluated and diagnosed by at least one medical specialist in rheumatology. Pulmonary arterial hypertension was defined as a pulmonary artery systolic pressure ≥ 40 mmHg by echocardiography. The group was divided as not severe when pressures ranged from 40 to 64 mmHg and severe, when pressures were ≥ 65 mmHg. RESULTS: The most common connective tissue disease associated with pulmonary arterial hypertension was diffuse scleroderma in 46% of cases. Eighty nine percent of patients were female. Time of evolution of the pulmonary hypertension was 18.8 ± 21.8 months. The distance walked in the six minute walk test was < 400 m both in patients with and without severe pulmonary hypertension. Fifty one percent of patients had pulmonary restriction. No differences in gas exchange parameters were observed between groups. Comparing echo cardio-graphic findings in patients with and without severe hypertension, the former had a higher frequency of right ventricular dilatation (85.7 and 52.3% respectively, p = 0.04), right ventricular hypertrophy (42.8 and 0% respectively, p = 0.02) and right ventricular hypokinesia (71.4 and 9.5% respectively p = < 0.01). CONCLUSIONS: Patients with severe pulmonary arterial hypertension associated to connective tissue diseases have more commonly dilated, hypertrophic and hypokinetic right ventricles.

Hipertensión arterial pulmonar por ecocardiografía en pacientes con enfermedades de tejido conectivo / Pulmonary hypertension associated with connective tissue diseases

Background: Pulmonary arterial hypertension is an important cause ofcomplica-tions amongpatients with connective tissue diseases. Aim: To describe the clinical and echocardiographic characteristics ofpatients with pulmonary hypertension associated with connective tissue diseases. Material and Methods: Retrospective, observational and descriptive study. We analyzed 35 patients with pulmonary hypertension associated with connective tissue diseases. All patients were evaluated and diagnosed by at least one medical specialist in rheumatology. Pulmonary arterial hypertension was defined as a pulmonary artery systolic pressure ≥ 40 mmHg by echocardiography. The group was divided as not severe when pressures ranged from 40 to 64 mmHg and severe, when pressures were ≥ 65 mmHg. Results: The most common connective tissue disease associated with pulmonary arterial hypertension was diffuse scleroderma in 46% of cases. Eighty nine percent of patients were female. Time of evolution of the pulmonary hypertension was 18.8 ± 21.8 months. The distance walked in the six minute walk test was < 400 m both in patients with and without severe pulmonary hypertension. Fifty one percent ofpatients had pulmonary restriction. No differences in gas exchange parameters were observed between groups. Comparing echocardio-graphic findings in patients with and without severe hypertension, the former had a higher frequency ofright ventricular dilatation (85.7 and 52.3% respectively, p = 0.04), right ventricular hypertrophy (42.8 and 0% respectively, p = 0.02) and right ventricular hypokinesia (71.4 and 9.5% respectively p = < 0.01). Conclusions: Patients with severe pulmonary arterial hypertension associated to connective tissue diseases have more commonly dilated, hypertrophic and hypokinetic right ventricles.