Pulmonary Capillary Hemangiomatosis With Severe Pulmonary Hypertension

Abstract

25-year-old man presented in 1998 with the complaint of progressive dyspnea on exertion and was admitted to hospital in August 1999. Cardiac catheterization revealed moderate pulmonary hypertension (PH; pulmonary artery pressure 61/33 mmHg). Congenital heart disease and left-sided heart failure were excluded by echocardiography. Collagen disease and liver disease were ruled out by negative results for the autoantibody test and liver function test. Furthermore, a perfusion lung scan did not show any perfusion defects, which excluded pulmonary thromboembolism as a cause of PH. 1 An acute challenge test with O2 inhalation or continuous infusion of epoprostenol (PGI2) under right heart catheterization showed a moderate decrease in pulmonary artery pressure. The patient then had a catheter implanted in superior vena cava and continuous infusion of epoprostenol was initiated, as well as home O2 therapy and an anticoagulation regimen with warfarin.Later, after the patient had been discharged, serial Doppler echocardiography revealed an increase in pulmonary pressure despite an increase in the dose of infused epoprostenol (maximum dose: 10 ng · kg -1 · min -1 ). The patient was readmitted in February 2000 because of exacerbation of the dyspnea on exertion. Physical examination showed an accentuated pulmonary component of the second heart sound and right ventricular heave. There was a prominent V wave of the jugular vein and hepatojugular reflux was observed. Marked hepatomegaly was also present, but without overt edema of the lower limbs. An electrocardiogram showed right ventricular hypertrophy with right heart strain and tachycardia. Right axis deviation and right ventricular hypertrophy had progressed between the initial and current admission (Fig 1). Circulation Journal Vol.67, September 2003Echocardiography showed an increase in pulmonary artery pressure and enlargement of both the right atrium and right ventricle with severe tricuspid regurgitation; left ventricular function was preserved. Measurement of arterial blood gases with inhalation of 15 L/min of O2 showed a pH of 7.437, CO2 tension of 24.4 mmHg and O2 tension of Circ J 2003; 67: 793 -795 (Received December 21, 2001; revised manuscript received April 8, 2002; accepted April 11, 2002 Pulmonary capillary hemangiomatosis (PCH) is a rare idiopathic lung disorder that occurs in young patients and leads to pulmonary hypertension (PH). It is difficult to diagnosis in the early stage and is often mistaken for primary PH; in almost all cases of PCH, the correct diagnosis is not made until autopsy. In the present case of PCH, the patient had severe pulmonary hypertension and died of respiratory failure. Pathologically, PCH is characterized by proliferation of benign thin-walled capillary sized blood vessels in the lung parenchyma. (Circ J 2003; 67: 793 -795)

Pulmonary Veno-occlusive Disease and Pulmonary Capillary Hemangiomatosis

“…Progressive dyspnea and fatigue characterize the clinical manifestations of both PVOD and PCH, which are thus typically misdiagnosed as pulmonary arterial hypertension (PAH) (1,10,(13)(14)(15). Patients may also have chronic cough (dry or pro-ductive), chest pain, syncope, or digital clubbing (1,6,15).…”

“…In PCH, the age range of affected patients is broad (2-71 years), with a mean age of 30 years, which is comparable to the age range of patients with PVOD. In contrast to PVOD, however, PCH occurs with equal frequency in both sexes in patients of any age (13)(14)(15). Median survival is 3 years from initial presentation, and, as in PVOD, death may occur only a few months after the onset of symptoms (13,15).…”

Familial Pulmonary Capillary Hemangiomatosis Early in Life

“…As in patient 1 and also in patient 2, PCH is often associated with PH [9]. Leading clinical symptoms like pulmonary distress and pulmonary hypertension in PCH have to be differentiated from other diseases like hematologic or immunologic diseases [10].…”

Pulmonary Capillary Hemangiomatosis Associated with CREST Syndrome: A Case Report and Review of the Literature

“…Although this nonspecific symptom is a clinical manifestation of PCH, it is also found with inflammatory alveolitis caused by the CREST syndrome, which was also included in our differential diagnosis. In the setting of primary pulmonary hypertension, the presence of hemoptysis, pleural effusion, elevated number of iron-laden bronchoalveolar macrophages, and an interstitial radiological pattern suggest PCH [2,4,5,6], though there has been at least one report in which pulmonary hypertension was absent in the presence of PCH [6], adding to the difficulty in diagnosing this rare disease. Retrospective analysis of a report by Humbert et al [4] demonstrated that the clinical presentation of PCH is indistinguishable from that of classic hypertensive pulmonary arteriopathy.…”