Long-Term Outcomes of Patients With Absent Pulmonary Valve Syndrome: 38 Years of Experience

Yong, Matthew S.; Yim, Deane; Brizard, Christian P.; Robertson, Terry; Bullock, Andrew; d’Udekem, Yves; Konstantinov, Igor E.

doi:10.1016/j.athoracsur.2014.01.035

Cited by 58 publications

(55 citation statements)

References 19 publications

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“…The potential for good outcomes was documented in a study by Yong et al in Australia over a 38-year period, which showed that the overall survival at 10 years was 81.4% ± 5.6%. (8) In conclusion, TOF with APVS is a rare condition compromising 3% of all TOF patients presenting to an African tertiary care centre, which correlates with the mainstream literature. The 22q11.2 deletion syndrome was suspected in 33% of patients but confirmed in a small number (13%), largely due to several of our patients presenting prior to the availability of routine testing.…”

Section: Resultssupporting

confidence: 61%

See 1 more Smart Citation

Tetralogy of Fallot with absent pulmonary valve syndrome: A 34-year African single centre experience

Mammen

Adams

Ntsinjana

et al. 2018

SAHJ

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Section: Resultssupporting

confidence: 61%

“…The overall survival in this study was 81.4% ± 5.6% at 10 years. (8) There is currently no literature emanating from Africa that has been published on TOF with APVS. The aim of the study was to review the characteristics and outcomes of patients presenting with this condition at a tertiary African referral hospital.…”

Section: Of Fallotmentioning

confidence: 99%

Tetralogy of Fallot with absent pulmonary valve syndrome: A 34-year African single centre experience

Mammen

Adams

Ntsinjana

et al. 2018

SAHJ

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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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“…The median age at time of repair ranges from 9 months to 1.4 years. 2,3 Even with repair during infancy, postoperative mortality rates range from 11% to 30%, and the 20-year survival rate is only 81%. 2 We present the case of a patient with only mild symptoms who underwent surgical repair for this combined condition as an adolescent.…”

mentioning

confidence: 99%

Unrepaired Tetralogy of Fallot with Absent Pulmonary Valve in a Mildly Symptomatic 16-Year-Old Boy

Drogalis-Kim¹,

Reemtsen²,

Reardon³

2016

Texas Heart Institute Journal

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T etralogy of Fallot (TOF) with absent pulmonary valve (PV) is a severe congenital heart disease found in only 3% to 6% of TOF patients.1 Survival into young adulthood, especially without repair, is quite rare. The median age at time of repair ranges from 9 months to 1.4 years.2,3 Even with repair during infancy, postoperative mortality rates range from 11% to 30%, and the 20-year survival rate is only 81%.2 We present the case of a patient with only mild symptoms who underwent surgical repair for this combined condition as an adolescent. Case ReportIn January 2015, a 16-year-old boy with no surgical history presented after the detection of a murmur. He and his parents were from Mexico, where his parents had been told that their son had a grave cardiac condition. Because of mortality rates said to exceed 50% in Mexico, they had been urged not to pursue surgical correction.The parents had noted no issues with their son's growth, development, or respiratory capabilities throughout his childhood and most of his adolescent years; he merely tired more rapidly than his peers and had occasional fluttering in his chest. He was taking no medications.On examination, the patient was acyanotic and in no distress. His pulse rate was 75 beats/min; blood pressure, 107/68 mmHg; and oxygen saturation, 100% on room air. For his age, his weight was in the 33rd percentile and his height in the 53rd. Cardiac examination revealed strong, symmetric pulses bilaterally; a grade 4/6 holosystolic murmur at the left upper sternal border with radiation throughout the thorax; and a 3/6 diastolic murmur at the left sternal border. The patient had no hepatomegaly or peripheral edema. A chest radiograph showed moderate cardiomegaly and dilation of the pulmonary artery (PA) (Fig. 1).A 2-dimensional transthoracic echocardiogram revealed severe pulmonary regurgitation and stenosis (Fig. 2). The main PA was dilated to 5.13 cm (Z score, 9.37) and the left PA to 3.72 cm (Z score, 10.36). The PV leaflets were extremely rudimentary, consistent with the physiology of TOF with absent PV (Fig. 3). The right ventricle was severely dilated with mildly reduced systolic function; left ventricular size and function were preserved. There was no pulmonary hypertension. The tricuspid regurgitation jet was 14 mmHg with a normal septal contour. A large, nonrestrictive ventricular septal defect had predominantly left-to-right flow. The PA enlargement was confirmed on cardiac magnetic resonance images (Figs. 4 and 5), which also revealed normal coronary anatomy and an extremely elevated right ventricular enddiastolic volume of 207.2 mL.

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Long-Term Outcomes of Patients With Absent Pulmonary Valve Syndrome: 38 Years of Experience

Cited by 58 publications

References 19 publications

Tetralogy of Fallot with absent pulmonary valve syndrome: A 34-year African single centre experience

Tetralogy of Fallot with absent pulmonary valve syndrome: A 34-year African single centre experience

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

Unrepaired Tetralogy of Fallot with Absent Pulmonary Valve in a Mildly Symptomatic 16-Year-Old Boy

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