Dilatation of the Pulmonary Arteries in Primary Pulmonary Hypertension

A rare case of extensive in situ central pulmonary artery thrombosis in primary pulmonary hypertension (PPH) is presented. The differentiation from chronic thromboembolic pulmonary arterial hypertension (CTEPH) is of paramount importance because of different therapeutic strategies. In this case, the presence of mural thrombus in the central pulmonary arteries on computed tomography made the distinction difficult. However, the possibility of in situ thrombosis was suggested on the basis of absence of other findings of CTEPH (abrupt narrowing/truncation of segmental arteries, variation in size of segmental vessels, arterial webs, mosaic attenuation, pulmonary infarcts, and dilated bronchial arteries), and this was confirmed on final pathology.

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Automated Morphologic Evaluation of Pulmonary Arteries in Primary Pulmonary Hypertension

Investigative Radiology ◽

10.1097/00004424-198612000-00002 ◽

1986 ◽

Vol 21 (12) ◽

pp. 906-909 ◽

Cited By ~ 2

Author(s):

LAWRENCE M. BOXT ◽

STUART RICH ◽

RUTHELLEN FRIED ◽

LIZELLEN LA FOLLETTE ◽

TAMAS SANDOR ◽

...

Keyword(s):

Pulmonary Hypertension ◽

Primary Pulmonary Hypertension ◽

Pulmonary Arteries

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PRIMARY PULMONARY HYPERTENSION

PEDIATRICS ◽

10.1542/peds.20.3.408 ◽

1957 ◽

Vol 20 (3) ◽

pp. 408-415

Author(s):

Harvey S. Rosenberg ◽

Dan G. McNamara

Keyword(s):

Pulmonary Hypertension ◽

Right Ventricular ◽

Ventricular Hypertrophy ◽

Primary Pulmonary Hypertension ◽

Pulmonary Arteries ◽

Right Ventricular Hypertrophy ◽

Vascular Change ◽

Progressive Dyspnea ◽

Peripheral Lung ◽

Secondary Pulmonary Hypertension

A case is reported of a 4-month-old infant in which the outstanding clinical features were failure to gain weight, cough, and progressive dyspnea. There was marked accentuation of the second pulmonic sound and no significant murmur. The electrocardiogram was interpreted as showing right ventricular hypertrophy and roentgenographically, there was unusual clarity of peripheral lung fields and prominence of hilar vessels. Cardiac catheterization demonstrated pulmonary hypertension and revealed no left to right intracardiac shunt. Anatomically, there was right ventricular hypertrophy and prominence of the medial layer of the small pulmonary arteries. This vascular change is indistinguishable from that seen in the normal newborn and certain varieties of secondary pulmonary hypertension. Although the clinical diagnosis can be suspected, the definitive anomaly can be determined only at post-mortem examination.

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The small pulmonary arteries in primary pulmonary hypertension and emphysema.

Thorax ◽

10.1136/thx.24.3.380-d ◽

1969 ◽

Vol 24 (3) ◽

pp. 380-380

Author(s):

P G Stovin

Keyword(s):

Pulmonary Hypertension ◽

Primary Pulmonary Hypertension ◽

Pulmonary Arteries

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2. An Autopsy Case of Sudden Death in a Boy with Primary Pulmonary Hypertension

Medicine Science and the Law ◽

10.1258/rsmmsl.45.4.361 ◽

2005 ◽

Vol 45 (4) ◽

pp. 361-363 ◽

Cited By ~ 3

Author(s):

Hitoshi Kawato ◽

Masahito Hitosugi ◽

Masahito Kido ◽

Tetsuo Yufu ◽

Toshiaki Nagai ◽

...

Keyword(s):

Pulmonary Hypertension ◽

Left Ventricle ◽

Sudden Death ◽

Ventricular Hypertrophy ◽

Primary Pulmonary Hypertension ◽

Pulmonary Arteries ◽

Histopathologic Examination ◽

Autopsy Case ◽

Plexiform Lesions ◽

Biventricular Failure

We report a rare autopsy case of sudden death due to primary pulmonary hypertension. A seven-year-old boy, who had been diagnosed with primary pulmonary hypertension at the age of four years, died suddenly. Forensic autopsy and histopathologic examination revealed extensive obstruction of small muscular pulmonary arteries by plexiform lesions and concentric intimal thickenings, compatible with primary pulmonary hypertension. We concluded that plexiform lesions of pulmonary arteries produced right ventricular hypertrophy and dilatation, decreased the preload of the left ventricle and subsequently led to biventricular failure. This autopsy and histopathologic examination suggested a possible pathophysiologic mechanism of sudden death due to primary pulmonary hypertension in a child.

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