“Absent” pulmonary valve with atrial septal defect and patent ductus arteriosus

Alpert, Bruce S.; Moore, H. Victor

doi:10.1007/bf02282748

Cited by 25 publications

(8 citation statements)

References 11 publications

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“…Associated atrial septal defects, an intact ventricular septum, and patent ductus arteriosus are reportedly rare [12][13][14]. In the present study, only 1 patient had tricuspid atresia, an intact ventricular septum, and patent ductus arteriosus.…”

Section: Discussioncontrasting

confidence: 52%

Echocardiography in the diagnosis of patients with absent pulmonary valve syndrome: a review study of 12 years

Pang

Lin

et al. 2015

Int J Cardiovasc Imaging

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Absent pulmonary valve syndrome (APVS) is a rare congenital heart disease that is easily misdiagnosed as tetralogy of Fallot (TOF). We herein discuss the echocardiographic features of APVS, compare its two subtypes, and clarify some differences between APVS and TOF. From July 1998 to October 2011, 31 patients diagnosed with APVS at Fuwai Hospital underwent echocardiography, computed tomography, or cardiac angiography. APVS was clinically categorized as either infant-type or child-type. We compared the echocardiographic similarities and differences between APVS and TOF and between the two subtypes of APVS. Although enlargement or aneurysmal dilatation was present in the main pulmonary artery (PA) and its branch in most patients, pulmonary dysplasia or even an absent left PA was found in a few patients. Four important echocardiographic features of APVS useful for distinguishing this syndrome from TOF were (1) absence of the pulmonary valve or presence of pulmonary valve dysplasia, (2) concurrent stenosis and regurgitation at the pulmonary annulus, (3) significant aneurysmal dilatation in the areas of the PAs, and (4) increased rather than decreased PA pressure. 10 patients had infant-type APVS and 21 had child-type APVS. Compared with child-type APVS, infant-type APVS was usually characterized by a lower oxygen saturation, more dilated main PA and right PA, lower aorta-PA ratio, higher diastolic PA pressure, and lower incidence of an absent left PA. Echocardiography is important for diagnosing APVS and distinguishing it from TOF. There are minimal differences in the echocardiographic features between infant-type and child-type APVS.

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Section: Discussioncontrasting

confidence: 52%

Echocardiography in the diagnosis of patients with absent pulmonary valve syndrome: a review study of 12 years

Pang

Lin

et al. 2015

Int J Cardiovasc Imaging

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“…The main difference between our case and that reported by Alpert and Moore [1] lies in the size of the atrial septal defect . In our case it was very small, amounting effectively to a patent foramen ovale, and in that respect it is similar to the experience reported by Ito et al .…”

contrasting

confidence: 54%

“Absent” pulmonary valve with ASD and PDA

1986

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“…In our case, the abnormal branching patterns of the pulmonary artery were not demonstrated on angiography, and this palliative procedure was apparently effective, suggesting that the aneurysmal dilatation or abnormal pattern of the peripheral pulmonary arteries was mild because of the patency of the ductus during fetal life. Although some newborns with absent pulmonary valve syndrome have been successfully treated with porcine valve insertion [1] or an aortic homograft conduit [6], little is known about the long-term outcome following these procedures. Chronic volume overload of the right ventricle in our case might induce right-sided heart failure over the long term [13].…”

Section: Discussionmentioning

confidence: 99%

“…Our patient also had a small ductus and recovered from severe cardiorespiratory failure. Some of the patients with a large ductus died during early infancy [5,12,14], and the others underwent ductus ligation during the neonatal period [1,6,7,9].…”

Section: Discussionmentioning

confidence: 99%

Absent Pulmonary Valve with Intact Ventricular Septum Presenting as Cardiorespiratory Failure at Birth

et al. 1997

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Absent pulmonary valve with intact ventricular septum is a rare congenital anomaly. Most cases are not diagnosed until years after birth, although several cases have been reported in the literature in which cardiorespiratory distress developed during the neonatal period. We present a patient who showed improvement after fairly conservative therapy to alleviate the pulmonary vascular resistance, with ultimate palliative surgery. The aneurysmal dilatation of the pulmonary artery was for the most part confined to the proximal portion of the trunk, possibly because the ductus arteriosus was patent. The literature is reviewed.

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“Absent” pulmonary valve with atrial septal defect and patent ductus arteriosus

Cited by 25 publications

References 11 publications

Echocardiography in the diagnosis of patients with absent pulmonary valve syndrome: a review study of 12 years

Echocardiography in the diagnosis of patients with absent pulmonary valve syndrome: a review study of 12 years

“Absent” pulmonary valve with ASD and PDA

Absent Pulmonary Valve with Intact Ventricular Septum Presenting as Cardiorespiratory Failure at Birth

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