Primary Pulmonary Obliterative Vascular Disease in Infants and Young Children

Husson, George S.; Wyatt, Tyree C.

doi:10.1542/peds.23.3.493

Cited by 16 publications

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“…The disease is Family Male Female Total thought to be transmitted as an autosomal Generations Reference disease, although it has been supposed to be I 0 U, 9]. We report two brothers with an almost 1 [13] dyspnoea on exercise since September 1986. 12 2 1 3 2 [14] he had a normal physical exercise capacity.…”

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confidence: 82%

Unexplained severe pulmonary hypertension in two brothers

Puolijoki¹,

Niemela²,

L³

1990

Eur Respir J

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Unexplained pulmonary hypertension of nearly simultaneous onset is reported in two brothers, aged 17 and 26 yrs. Echocardiography revealed right ventricular hypertrophy and dilatation, paradoxical septal motion and an enlarged main pulmonary artery. In the right catheterization highly elevated pulmonary arterial pressures (107/58 and 84/46 mmHg) were seen. Doppler echocardiography showed significant leakage of the pulmonary and tricuspid valves in the younger brother, who died suddenly three weeks later. An initial decline in pulmonary arterial systolic pressure was achieved in the older brother by drug therapy. Eight months later, however, the pressure had risen to the pretreatment level. Dyspnoea increased and the patient underwent heart-lung transplantation but subsequently died. In the family study a third brother, father and mother were healthy.

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confidence: 82%

Unexplained severe pulmonary hypertension in two brothers

Puolijoki¹,

Niemela²,

L³

1990

Eur Respir J

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Early Onset of Pulmonary Vascular Obstruction With Atrial Septal Defect

Herbert¹,

Goyal²,

Wadhwani³

1969

Arch Pediatr Adolesc Med

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TODAY the diagnosis of atrial septal defect (ASD) in childhood no longer engenders anxiety or even a sense of urgency. The repair of an uncomplicated ASD is associated with a low and quite generally acceptable mortality.1-4 That there is no aspect of urgency is attributable to a preponderant evidence that ASD is benign in childhood.5-10 Indeed, not only may there be a remarkable longevity associated with this lesion 11-16 but the patients attaining such longevity commonly do not demonstrate significant pulmonary hypertension.12-16 This is not to say, however, that a certain morbidity and mortality has not been ascribed to ASD in infancy and childhood. Hastreiter et al10 described 13 cases in which congestive heart failure secondary to ASD was considered the primary cause of death. All of these patients developed a fulminant clinical picture prior to 18 months of age.Only three patients were older than 6 months. Taussig 17 described an additional overload of the right ventricle producing right heart failure presumably precipi¬ tated by an effort produced elevation in left heart pressure. These episodes re¬ sponded to therapy and tended to di¬ minish with advancing age.It is the consensus then that ASD is,

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Vermehrung der Erythrocyten

Weippl¹

1967

Erkrankungen Der Stützgewebe Erkrankungen Des Blutes Und Der Blutbildenden Organe

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Primary Pulmonary Obliterative Vascular Disease in Infants and Young Children

Abstract: Extensive clinical studies of three children with primary pulmonary obliterative vascular disease are reported. Two were siblings. The pathology is described from two necropsies. On the basis of the present study and a review of the literature a concept of the pathogenesis is developed.

Cited by 16 publications

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Unexplained severe pulmonary hypertension in two brothers

Unexplained severe pulmonary hypertension in two brothers

Early Onset of Pulmonary Vascular Obstruction With Atrial Septal Defect

Vermehrung der Erythrocyten

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