Idiopathic pulmonary hypertension

Farrar, J. F.

doi:10.1016/0002-8703(63)90079-6

Cited by 17 publications

(15 citation statements)

References 41 publications

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“…Rosenberg (1964) considered that all instances of primary pulmonary hypertension were caused by multiple pulmonary embolization. Farrar (1963) Cases 3 and 4 suffer from a severe degree of primary pulmonary hypertension. In the families so far reported, female members have usually been predominantly involved (Table II).…”

Section: Discussionmentioning

confidence: 99%

“…In the absence of any known aetiological. factor (Farrar, 1963;Berthrong and Cochran, 1955), pulmonary hypertension in these children can be assumed to be primary. Five members of the present family suffer from lymphoedema of the feet and legs.…”

Section: Discussionmentioning

confidence: 99%

“…Rosenberg (1964) considered that all instances of primary pulmonary hypertension were caused by multiple pulmonary embolization. Farrar (1963) brought forward the possible involvement of some hypersensitive immune mechanism. Kingdon et al (1966) gave as a possibility the presence of a genetically determined hypercoagulable state, leading to thrombi formation and embolization of the pulmonary vascular tree.…”

Section: Discussionmentioning

confidence: 99%

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Primary pulmonary hypertension, cerebrovascular malformation, and lymphoedema feet in a family.

Avasthey¹,

Roy²

1968

Heart

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Since Clarke et al. (1927) described primary pulmonary hypertension in two sisters, several families with more than one member involved have been reported (Lange, 1948;Dresdale, Michtom, and Schultz, 1954;van Epps, 1957; Coleman, Edmunds, and Tregillus, 1959;Fleming, 1960;Cahen et al., 1961;van Bogaert et al., 1961;Boiteau and Libanoff, 1963;Melmon and Braunwald, 1963;Parry and Verel, 1966;Kingdon et al., 1966). The present family is interesting because its several members suffered from more than one disease in common. CASE REPORTSThe family first came under our observation in 1962. The grandparents were not known to have suffered from heart disease or lymphoedema. Sibs of the parents were described by them to be healthy. The family tree is shown in Fig. 1. Case 1. This 38-year-old woman, the mother of the other patients to be described, had had two episodes of high fever, and redness and swelling over both feet and legs, at the ages of 13 and 15 years, respectively. Both attacks lasted two days each. Ever since then she had noticed swelling over both feet. Physical examination showed her to be a healthy-looking woman with nonpitting oedema over both feet and some pitting oedema over the shins. There was no clubbing or cyanosis. Peripheral pulses were normal. A loud systolic bruit was audible over both temporal regions of the skull and was conducted down the carotids. Fundi and the central nervous system were normal. Cardiovascular and respiratory systems and abdomen were normal. Skiagram of the chest showed a rounded small shadow in the left infraclavicular region, which was thought to be an arrested Koch's lesion. Heart was normal in size. Urinalysis and routine blood investigations were normal, as was a 12-lead electrocardiogram. No acid-fast organisms were grown from the laryngeal swab. Case 2. The eldest child of Case 1 was 20 at the time of emination. His parents thought he had always been of low intelligence. He gave a history of repeated attacks of painful red swelling over both feet since the age of 8. He was a well-developed boy of subnormal intelligence; slight non-pitting oedema was present over the feet; heart, lungs, and abdomen were normal. A loud systolic bruit was heard all over the head and along the carotid vessels. Optic fundi were normal and there was no neurological deficit. Thoracic x-ray examination and electrocardiogram were normal. A right-sided carotid angiogram revealed a large extracranial arteriovenous malformation over the postero-superior parietal region (Fig. 2). The superficial temporal and occipital branches of the external carotid artery were tortuous and dilated and fed the malformation. No dye was seen in the internal carotid artery or its branches.Case 3. He was 16 years of age and had a history of attacks of fever and redness and swelling over the feet since the age of 8. He was found to have lymphoedema over both feet. The heart was clinically enlarged, with

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

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Primary pulmonary hypertension, cerebrovascular malformation, and lymphoedema feet in a family.

Avasthey¹,

Roy²

1968

Heart

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“…Theories of the etiology of primary pulmonary hypertension have been reviewed by Farrar (1963). The pressures in the pulmonary artery and the aorta before birth are approximately equal and the vessels are histologically similar.…”

Section: Discussionmentioning

confidence: 99%

Familial primary pulmonary hypertension.

Parry¹,

Vérel²

1966

Heart

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“…Idiopathic pulmonary arterial hypertension (PAH) is characterized by the progressive rise in pulmonary artery pressure, leading to right ventricular failure and death (11-14). Recent published studies have demonstrated that peak systolic and diastolic TDI velocities were reduced in adult patients with idiopathic PAH compared with normal controls in both the interventricular septum as well as the tricuspid annulus, whereas no significant differences were noted between idiopathic PAH patients and controls in the mitral annulus velocities (15).…”

mentioning

confidence: 99%

Tissue Doppler Imaging Predicts Adverse Outcome in Children with Idiopathic Pulmonary Arterial Hypertension

Takatsuki

Nakayama

Jone

et al. 2012

The Journal of Pediatrics

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Objective To evaluate the clinical utility of tissue Doppler imaging (TDI) in assessment of disease severity and prognostic value in children with idiopathic pulmonary arterial hypertension (PAH). Study design A prospective study was performed to evaluate TDI velocities (Sm; systolic velocity, Em; early diastolic velocity, Am; late diastolic velocity), brain natriuretic peptide (BNP), New York Heart association (NYHA) functional class, and hemodynamics in 51 children (mean age;11.6 years) with idiopathic PAH. Fifty-one healthy children with comparable demographics served as controls. Results Em, Em/Am ratio, and Sm at mitral annulus, septum, and tricuspid annulus in PAH were significantly reduced compared with controls. Tricuspid Em had significant inverse correlations with plasma BNP levels (r=−0.60, p<0.001), right ventricular end-diastolic pressure (r=−0.79, p<0.001), and mean pulmonary arterial pressure (r=−0.67, p<0.001). Statistically significant differences were observed in tricuspid Em between NYHA functional class II versus combined III and IV (mean and standard deviation; 11.9+/−4.2 cm/s versus 8.2+/−3.6 cm/s, respectively, p=0.002). Cumulative event-free survival rate was significantly lower when tricuspid Em was ≤ 8 cm/s (log-rank test, p<0.001) Conclusions Tricuspid Em velocity correlated with NYHA functional class as disease severity and may serve as a useful prognostic marker in children with idiopathic PAH. The present study is the initial report to evaluate TDI velocities against mid-term outcome variables in a relatively large pediatric PAH population.

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Idiopathic pulmonary hypertension

Cited by 17 publications

References 41 publications

Primary pulmonary hypertension, cerebrovascular malformation, and lymphoedema feet in a family.

Primary pulmonary hypertension, cerebrovascular malformation, and lymphoedema feet in a family.

Familial primary pulmonary hypertension.

Tissue Doppler Imaging Predicts Adverse Outcome in Children with Idiopathic Pulmonary Arterial Hypertension

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