An autopsy case of pulmonary capillary hemangiomatosis without evidence of pulmonary hypertension

Umezu, Hajime; Naito, Makoto; Yagisawa, Kumiko; Hattori, Akira; Aizawa, Yoshifusa

doi:10.1007/s004280100465

Cited by 33 publications

(24 citation statements)

References 21 publications

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“…Havlik et al 9 found incidental PCH-like foci in 5.7% of autopsy cases without any symptoms of PH; the distribution of the foci was local and the patients were old men, so that may be a different entity from PCH, which affects young adults of both sexes and has diffuse histopathological changes. Umezu et al 10 reported an autopsy case of PCH without any evidence of PH in which proliferation of capillaries was observed in the alveolar and bronchial walls, but not in the vascular walls; however, most patients with PCH, including the present case, have PH and related symptoms because of the diffuse proliferation of capillaries. Because PCH is a rare disorder, it is not clear at this point whether PCH without PH represents an early stage of PCH or is a different class of PCH.…”

Section: Discussionmentioning

confidence: 48%

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: Discussionmentioning

confidence: 48%

Pulmonary Capillary Hemangiomatosis With Severe Pulmonary Hypertension

Itō¹,

Ichiki²,

Ohi³

et al. 2003

Circ J

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“…Haemangiomatosis-like foci and distinct local manifestations can cause diagnostic difficulties [18,19]. PH (in certain instances PPH) is generally (but not in all cases) regarded as a 'sine qua non' prerequisite of the diagnosis of PCH [4,27,42,47,49,52]. The WHO classification [39] differentiates five categories of PH: (1) pulmonary arterial hypertension, (2) pulmonary venous hypertension, (3) PH associated with disorders of the respiratory system and/or hypoxaemia, (4) PH due to chronic thrombotic and/or embolic disease, and (5) PH due to disorders directly affecting the pulmonary vasculature [13,39].…”

Section: Discussionmentioning

confidence: 99%

Pulmonary capillary haemangiomatosis in children and adolescents: report of a new case and a review of the literature

et al. 2004

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Pulmonary capillary haemangiomatosis (PCH) in childhood is a rarity, characterised by the uncontrolled proliferation of pulmonary microvessels which may invade pulmonary, bronchial and vascular structures, resulting in diffuse alveolar haemorrhage, manifesting clinically in haemoptysis, dyspnoea and symptoms of pulmonary hypertension (PH). A 14-year-old boy with some particular features (pericardial effusion and thrombocytopenia) is presented and 14 paediatric/adolescent cases from the literature are surveyed. The diagnostic problems and difficulties are discussed, including the importance of imaging (high-resolution CT) and histopathological studies, with the aim of providing a clear-cut distinction of PCH from other conditions such as primary PH (PPH). The literature data can be regarded as ambiguous: both similarities and relatively sharp distinctions between PCH and PPH are to be found. New developments in the field of genetics are also discussed. The early coexistence of PCH and other (vascular) disorders and associations, involving focal or diffuse, disseminated forms is summarised briefly. Conclusion. The diagnosis of this progressive disorder may lead to effective therapy. Treatment possibilities include the rapidly evolving field of anti-angiogenic therapy, but at present lung transplantation is universally accepted as the final definitive treatment for pulmonary capillary haemangiomatosis.

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“…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.…”

Section: Discussionmentioning

confidence: 99%

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

et al. 2010

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This is a report of fatality immediately after administration of epoprostenol. The patient was previously diagnosed with CREST syndrome and associated interstitial lung disease. She developed worsening pulmonary hypertension and was clinically diagnosed with pulmonary veno-occlusive disease. The patient developed flash pulmonary edema and arrested after administration of low-dose epoprostenol in the intensive care unit. An autopsy revealed the patient suffered from pulmonary capillary hemangiomatosis. We review our case and what is known about this rare disease.

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An autopsy case of pulmonary capillary hemangiomatosis without evidence of pulmonary hypertension

Cited by 33 publications

References 21 publications

Pulmonary Capillary Hemangiomatosis With Severe Pulmonary Hypertension

Pulmonary Capillary Hemangiomatosis With Severe Pulmonary Hypertension

Pulmonary capillary haemangiomatosis in children and adolescents: report of a new case and a review of the literature

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

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