Pulmonary Capillary Hemangiomatosis

Lippert, John L.; White, Charles S.; Cameron, Erik W.; Sun, Chen; Liang, Xiayuan; Rubin, Lewis J.

doi:10.1097/00005382-199801000-00012

Cited by 43 publications

(29 citation statements)

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“…In the present case, an acute challenge test of epoprostenol decreased the pulmonary artery pressure and therefore continuous infusion of epoprostenol was instituted; however, both the dyspnea on exertion and the pulmonary artery pressure gradually worsened. The same clinical course was reported by Lippert et al 3 It is reported that epoprostenol may cause pulmonary edema in patients with pulmonary occlusive diseases such as PCH and pulmonary veno-occlusive disease, 7,8 but we did not observe pulmonary edema in this case, which suggests that epoprostenol may have been effective in the early stage of PCH.…”

Section: Discussionsupporting

confidence: 89%

“…2 Most of the patients are 20-40 years of age, as was the present case, and the earliest symptom is a gradual onset of shortness of breath on exertion. 3 Because patients with primary PH also frequently complain of the same symptom, PCH is often misdiagnosed. Although hemoptysis is a symptom of PCH, it is observed in only 30% of the patients and is non-specif- ic.…”

Section: Discussionmentioning

confidence: 99%

“…Although hemoptysis is a symptom of PCH, it is observed in only 30% of the patients and is non-specif- ic. 3 Therefore, careful follow-up by chest radiography has been recommended because PCH typically has a characteristic radiographic feature of a diffuse coarse reticulonodular pattern. Computed tomography usually shows a mosaic pattern of attenuation of pulmonary parenchyma and lobular ground-glass opacification in the area of increased pulmonary perfusion.…”

Section: Discussionmentioning

confidence: 99%

“…Although a case of successful treatment of PCH with interferon has been reported, 6 the best treatment for PCH is bilateral lung transplantation. 3 Although it is difficult, diagnosis in the early stage is -2a important. In the present case, an acute challenge test of epoprostenol decreased the pulmonary artery pressure and therefore continuous infusion of epoprostenol was instituted; however, both the dyspnea on exertion and the pulmonary artery pressure gradually worsened.…”

Section: Discussionmentioning

confidence: 99%

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Pulmonary Capillary Hemangiomatosis With Severe Pulmonary Hypertension

Itō¹,

Ichiki²,

Ohi³

et al. 2003

Circ J

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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)

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Section: Discussionsupporting

confidence: 89%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

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Pulmonary Capillary Hemangiomatosis With Severe Pulmonary Hypertension

Itō¹,

Ichiki²,

Ohi³

et al. 2003

Circ J

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“…287,[359][360][361][362][363] Sildenafil was approved by the FDA for group 1 PAH in 2005; its intravenous formulation was approved in 2009. 387 Early evaluation of sildenafil in 14 children with PAH showed an increase in 6MWD from 278±114 to 443±107 m over 6 months (P=0.02); at 12 months, the distance walked was 432±156 m (P=0.005) and was associated with a decrease in mPAP and PVR. 364 In several small studies of children with PPHN, IPAH, and PH associated with CHD, sildenafil has been shown to improve exercise capacity and hemodynamics.…”

Section: Pde Inhibitorsmentioning

confidence: 94%

Pediatric Pulmonary Hypertension

Abman¹,

Hansmann²,

Archer³

et al. 2015

Circulation

916

405

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Abstract-Pulmonary hypertension is associated with diverse cardiac, pulmonary, and systemic diseases in neonates, infants, and older children and contributes to significant morbidity and mortality. However, current approaches to caring for pediatric patients with pulmonary hypertension have been limited by the lack of consensus guidelines from experts in the field. In a joint effort from the American Heart Association and American Thoracic Society, a panel of experienced clinicians and clinician-scientists was assembled to review the current literature and to make recommendations on the diagnosis, evaluation, and treatment of pediatric pulmonary hypertension. This publication presents the results of extensive literature reviews, discussions, and formal scoring of recommendations for the care of children with pulmonary hypertension. Key Words: AHA Scientific Statements ◼ bronchopulmonary dysplasia ◼ congenital diaphragmatic hernia ◼ congenital heart disease ◼ genetics ◼ persistent pulmonary hypertension of the newborn ◼ sickle cell disease © 2015 by the American Heart Association, Inc., and the American Thoracic Society.Circulation is available at http://circ.ahajournals.org DOI: 10.1161/CIR.0000000000000329 †Deceased. The American Heart Association and the American Thoracic Society make every effort to avoid any actual or potential conflicts of interest that may arise as a result of an outside relationship or a personal, professional, or business interest of a member of the writing panel. Specifically, all members of the writing group are required to complete and submit a Disclosure Questionnaire showing all such relationships that might be perceived as real or potential conflicts of interest.This document was approved by the American Heart Association Science Advisory and Coordinating Committee on May 12, 2015, the American Heart Association Executive Committee on July 22, 2015, and the American Thoracic Society on July 24, 2015.The online-only Data Supplement is available with this article at http://circ.ahajournals.org/lookup/suppl/doi:10.1161/CIR.0000000000000329/-/DC1. The American Heart Association requests that this document be cited as follows: Abman SH, Hansmann G, Archer SL, Ivy DD, Adatia I, Chung WK, Hanna BD, Rosenzweig EB, Raj JU, Cornfield D, Stenmark KR, Steinhorn R, Thébaud B, Fineman JR, Kuehne T, Feinstein JA, Friedberg MK, Earing M, Barst RJ, Keller RL, Kinsella JP, Mullen M, Deterding R, Kulik T, Mallory G, Humpl T, Wessel DL; on behalf of the American Heart Association Council on Cardiopulmonary, Critical Care, Perioperative and Resuscitation, Council on Clinical Cardiology, Council on Cardiovascular Disease in the Young, Council on Cardiovascular Radiology and Intervention, Council on Cardiovascular Surgery and Anesthesia, and the American Thoracic Society. Pediatric pulmonary hypertension: guidelines from the American Heart Association and American Thoracic Society. Circulation. 2015;132:2037-2099 Copies: This document is available on the World Wide Web site of the American Heart Associat...

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Pulmonary edema complicating prostacyclin therapy in pulmonary hypertension associated with scleroderma: A case of pulmonary capillary hemangiomatosis

Gugnani

Pierson

VANDERHEIDE

et al. 2000

Arthritis & Rheumatism

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Continuous intravenous infusion of prostacyclin is an effective treatment for primary pulmonary hypertension (PPH), and has recently been shown to be of benefit in PH associated with scleroderma (SSc). Pulmonary capillary hemangiomatosis (PCH) is a rare cause of PPH. Prostacyclin therapy has been complicated by pulmonary edema in cases of PCH. We describe a case of PH associated with limited SSc, where treatment with prostacyclin was complicated by pulmonary edema. Autopsy revealed PCH as the pathologic basis for the PH. There were no clinical features of PCH prior to initiation of vasodilator therapy, illustrating the potential difficulty in establishing the diagnosis. This is the first reported case of PCH in SSc.

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Pulmonary Capillary Hemangiomatosis

Cited by 43 publications

References 0 publications

Pulmonary Capillary Hemangiomatosis With Severe Pulmonary Hypertension

Pulmonary Capillary Hemangiomatosis With Severe Pulmonary Hypertension

Pediatric Pulmonary Hypertension

Pulmonary edema complicating prostacyclin therapy in pulmonary hypertension associated with scleroderma: A case of pulmonary capillary hemangiomatosis

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