Chronic thromboembolic pulmonary hypertension (CTEPH) leads to increased vascular resistance and progressive right heart failure resulting from occlusion of proximal pulmonary arteries by fibrotic intravascular material. The elective surgical treatment for CTEPH is pulmonary endarterectomy (PEA), which involves removal of the chronic thromboembolic material from the entire pulmonary arterial tree. This study aimed to verify the effects of acute and subacute postoperative rehabilitation on the functional exercise capacity, dyspnea, and quality of life (QoL) of patients with CTEPH undergoing PEA. This was a systematic review of seven primary databases. At the end of the selection process, five documents were included in the final analysis. Three of these five studies were conducted in Italy, one in Korea, and one in Germany. Of 204 patients, 95 (47%) were male. This systematic review determined that in patients with CTEPH pre-PEA, the 6-minute walk distance (6MWD) ranged between 284.7 m and 371.95 m and that at 6 to 12 weeks post-PEA, after having attended postoperative rehabilitation, the 6MWD was 434.1 m to 483.6 m. The Short Form-36 Health Survey (SF-36) questionnaire was administered to evaluate QoL 3 and 22 weeks after PEA. At 22 weeks, the SF-36 physical component summary score improved from baseline (42.78±18.37 points) by 19.55±19.42 points (p=0.001), and the SF-36 mental component summary score improved from baseline (55.76±23.94 points) by 6.36±20.44 points (p=0.137).
Pulmonary tumor thrombotic microangiopathy (PTTM) is a rare but fatal complication of cancer and causes pulmonary hypertension and acute/subacute right heart failure. PTTM is most commonly associated with gastric cancer and more rarely associated with pancreatic cancer. We report a case of progressive right heart failure associated with clinically diagnosed pancreatic cancer, suggesting PTTM.
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Cardiovascular beriberi is caused by thiamine deficiency and usually presents as high cardiac output failure associated with predominantly right-sided heart failure and rapid recovery after treatment with thiamine. Because of its rarity in developed countries, the diagnosis can often be delayed and missed. We recently experienced a case of cardiovascular beriberi with pulmonary hypertension which successfully treated with thiamine infusion. A 50-year-old man with chronic heavy alcoholics was refered to our department for dyspnea with mental change. Echocardiography showed marked right ventricular (RV) dilatation and flattening of the interventricular septum with a D-shaped deformation of the left ventricle. Moderate tricuspid valve regurgitation was found and estimated RV systolic pressure was 52 mm Hg. Because of his confused mentality and history of chronic alcohol intake, neurological disorder due to thiamine deficiency was suspected and intravenous thiamine was administered and he continuously received a daily dose of 100 mg of thiamine. Follow up echocardiography showed marked reduction of RV dilatation and improvement of a D-shaped deformation of the left ventricle. He finally diagnosed as cardiovascular beriberi on the basis of dramatic response to intravenous thiamine. Thiamine deficiency can cause reversible pulmonary hypertension, and can still be encountered in the clinical setting. Thus high index of suspicion is critically needed for diagnosis.
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Pulmonary hypertension is an increase in blood pressure in the pulmonary artery, pulmonary vein or pulmonary capillaries. Depending on the cause, pulmonary hypertension can be a severe disease with markedly decreased exercise tolerance and right-sided heart failure. Pulmonary hypertension can present as one of five different types: arterial, venous, hypoxic, thromboembolic, or miscellaneous. Chronic obstructive pulmonary disease with severe pulmonary hypertension is a rare disease. A 52-year-old man presented with a complaint of aggravating dyspnea. The mean pulmonary arterial pressure was 61.5 mmHg by Doppler echocardiogram. The patient was prescribed diuretics, digoxin, bronchodilator, sildenafil, bosentan and an oxygen supply. However, he ultimately died of cor pulmonale. Thus, diagnosis and early combination therapy are important.