Anatomic Variability and Outcome in Prenatally Diagnosed Absent Pulmonary Valve Syndrome

Szwast, Anita; Tian, Zhenjun; McCann, Margaret; Soffer, Debbra; Combs, Jill; Donaghue, Denise; Rychik, Jack

doi:10.1016/j.athoracsur.2014.03.002

Cited by 36 publications

(56 citation statements)

References 25 publications

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“…Since then, most reports of AVPS have been case reports [3][4][5][6][7]. Although some papers have reported the outcomes and fetal characteristics of this disease [8,9], echocardiographic investigations of large patient samples are still lacking. Echocardiography is effective for the diagnosis of both TOF and APVS.…”

Section: Introductionmentioning

confidence: 99%

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: Introductionmentioning

confidence: 99%

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 congenital heart defect usually associated with tetralogy of Fallot . Absent (or rudimentary) pulmonary valve leaflets cause severe dilation of the pulmonary trunk and branches in the setting of valvar stenosis and severe insufficiency.…”

Section: Discussionmentioning

confidence: 99%

“…The presence of a large DA results in severe respiratory distress and signs of congestive heart failure early after birth which can be improved by early surgical closure of the DA . There are 15 reported cases of prenatally diagnosed APVS/IVS . Significant tricuspid valve pathology was present in 11 (9 atretic, 1 stenotic, and 1 severely regurgitant) and was associated with poor outcomes (20% transplant‐free survival, all with single ventricle physiology) .…”

Section: Discussionmentioning

confidence: 99%

“…There are 15 reported cases of prenatally diagnosed APVS/IVS . Significant tricuspid valve pathology was present in 11 (9 atretic, 1 stenotic, and 1 severely regurgitant) and was associated with poor outcomes (20% transplant‐free survival, all with single ventricle physiology) . Of the 4 remaining cases without tricuspid valve disease, two resulted in termination of pregnancy and two were live born.…”

Section: Discussionmentioning

confidence: 99%

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Prenatal physiologic findings and postnatal advanced imaging in the management of absent pulmonary valve syndrome with intact ventricular septum

et al. 2018

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Absent pulmonary valve syndrome is a congenital heart defect usually associated with tetralogy of Fallot. Rarely, absent pulmonary valve syndrome can occur independently of tetralogy of Fallot and presents with an intact ventricular septum and a patent ductus arteriosus. This case report describes the prenatal diagnosis of absent pulmonary valve syndrome with intact ventricular septum via echocardiogram and the postnatal management of the resulting physiologic effects secondary to this rare congenital heart disease.

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“…In the early '80s and '90s as well as in the early 2000s, it was believed that the later surgery in the infancy, the higher the odds of a good postoperative prognosis in patients with APVS 11,12 . Due to the fact that this is a very rare defect, the literature available so far has a small database of postoperative results, and in the last decade there were only six reports comprehensive publications based on the analysis of a dozen cases (12 -52) [13][14][15][16][17][18] . The authors analyzed the width of the lung branch, the presence of DA (ductus arteriosus), the association of defects with chromosomal abrasions (usually di George syndrome), a type of modification of cardiac surgery.…”

confidence: 99%

Absent Pulmonary Valve Syndrome – Special Prenatal Care and Early Surgery in Obstetric/Cardiac Center - A New Hope for Postnatal Survivors?

Zych-Krekora

Krekora

Kopala

et al. 2017

Prenatal Cardiology

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Introduction: APVS (Absent Pulmonary Valve Syndrome) is a rare congenital heart disease. Its incidence according to The Polish National Registry of Fetal Cardiac Pathology in years 2004 - 2016 was 0.6%.This disease is caused by the absence or the residual pulmonary artery valve resulting in significant dilation of the pulmonary trunk and its branches. In utero deaths are reported. After birth the major problem is respiratory failure and high preoperative and postoperative mortality. Material and methods: In 1995 to 2016, 11 fetuses with APVS were diagnosed in our unit, at the average 27,5 weeks of gestation (min. 18.5- max 37.1 weeks of gestation). Two groups were analysed in this series of cases: “Old one” by 2011 (n = 6) and “New one” since 2011 (n = 5) and perinatal care as well as survival were compared. We analysed the fetal echo results, perinatal care including transplacental digoxin and steroids treatment in NEW group, the longevity of the pregnancy and neonatal/infants outcome. Results: In Old group the average day of cardiac surgery was day 91st after birth (max. 161) and the survival was 50% . In the New group the average surgery day was 41st day and the postoperative survival was 60%, however there was no statistical significance ( p > 0,05). Conclusions: There is no single parameter from prenatal life in foetuses with APVS which may allow to predict the positive outcome meaning neonatal survival. However optimal perinatal care (early detection of defect, transplacental digoxin at least 3-4 weeks, steroids, no preterm delivery, on-time delivery, postnatal care in tertiary center) and relatively early cardiac surgery may have combined impact on the improvement of survival after prenatal diagnosis of APVS, however more data are necessary to prove this hypothesis.

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Anatomic Variability and Outcome in Prenatally Diagnosed Absent Pulmonary Valve Syndrome

Cited by 36 publications

References 25 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

Prenatal physiologic findings and postnatal advanced imaging in the management of absent pulmonary valve syndrome with intact ventricular septum

Absent Pulmonary Valve Syndrome – Special Prenatal Care and Early Surgery in Obstetric/Cardiac Center - A New Hope for Postnatal Survivors?

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