Superior vena cava obstruction and pulmonary artery involvement as rare findings of immunoglobulin G4-related disease.

We describe a case of immunoglobulin G4-related lung disease presenting as chronic pulmonary hypertension with involvement of right pulmonary artery and superior vena cava. Immunoglobulin G4- related disease is a rare systemic sclerosing disease with autoimmune entity that causes fibrotic, often mass-like manifestations that variably affect different organ systems and can be mistaken with other disorders. Timely diagnosis requires awareness on the part of clinicians and pathologists as well as radiologists to the variable manifestations of this newly recognized disorder. .

Hemodynamic and symptomatic improvement after delayed thrombolysis with Reteplase in a patient with massive bilateral pulmonary emboli.

Most patients surviving the acute phase of pulmonary embolism will recover with no residue. But, 2-4% of patients will progress to chronic thromboembolic pulmonary hypertension. In this group, usually a 'Honey moon' period is seen but a few may show progression with ongoing symptoms despite medical treatment. In this case report, we review a patient in whom delayed thrombolytic therapy was administered due to progressive symptoms after 21 days. Her condition was stabilized. The early posttreatment computed tomographic pulmonary angiography (CTPA) showed incomplete resolution, but after 6 months she was functional class I with a normal CTPA and echocardiography.

Late relapse of multiple myeloma presenting as a right atrial mass.

Intracardiac involvement is a rare presentation of relapsed multiple myeloma. We present a case of lobulated mass in the wall of the right atrium, with extension to the inferior vena cava and obliteration and encasement of the interatrial septum in a 57-year-old man, a known case of multiple myeloma, with progressive exertional dyspnea and anasarca.

Oral l-citrulline malate in patients with idiopathic pulmonary arterial hypertension and Eisenmenger Syndrome: a clinical trial.

BACKGROUND AND PURPOSE: Citrulline is an amino acid which is produced by the urea cycle and also a precursor for NO, that is, a vasodilator for normal function of pulmonary vasculature. Thereby, enhancing l-citrulline malate in patients with idiopathic pulmonary arterial hypertension and those with congenital heart disease identified as Eisenmenger Syndrome results in reduction of pulmonary hypertension. METHODS AND SUBJECTS: In this clinical trial before and after study, we assigned 25 patients with arterial pulmonary hypertension (idiopathic or Eisenmenger Syndrome) to receive l-citrulline malate 1g three times daily for two weeks. The primary measurement was the change in exercise capacity, as considered as a result of the total distance walked in six minutes, from baseline to week 2. We also assessed mean pulmonary artery pressure, the change in the quality of life, and the change in pro-brain natriuretic peptide (BNP) level. The study was not powered to evaluate mortality. RESULTS: The mean walking distance in six minutes was significantly increased by about 44m (p=0.005) after receiving l-citrulline malate. Mean pulmonary artery pressure significantly reduced from 83.34mmHg before receiving l-citrulline malate to 79.1mmHg after that (p=0.01). All dimensions of the quality of life had statistical differences after receiving l-citrulline malate except limit due to physical health, limit due to emotional health and social functioning (p>0.05). Finally, pro-BNP difference was not statistically significant (p=0.9). CONCLUSION: l-Citrulline malate improves the distance walk in six minutes and also the quality of life of patients with idiopathic arterial pulmonary hypertension and Eisenmenger Syndrome and also reduced mean arterial pulmonary hypertension.

Pericardial hydatid cyst in oblique sinus, obstructing all pulmonary veins: a rare presentation.

Hydatid cyst of the heart is an uncommon presentation of hydatidosis. We present a case of pericardial hydatid cyst in pericardial oblique sinus with extension to posterior wall of left atrium (LA), occluding all pulmonary vein ostia in a 35 year-old female with progressive dyspnea and severe orthopnea.