Absent pulmonary valve syndrome.☆Surgical and clinical outcome with long-term follow-up

Nørgaard, Martin Agge; Alphonso, Nelson; Newcomb, Andrew; Cochrane, Andrew

doi:10.1016/j.ejcts.2006.01.050

Cited by 40 publications

(23 citation statements)

References 17 publications

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“…Norgaard et al inserted monocusp pulmonary valves from a variety of materials in 39% of the total corrective surgeries performed on 36 patients with APVS and TOF. They found no significant survival differences between the groups having no pulmonary valve, monocusp valves, and homograft conduits …”

Section: Discussionmentioning

confidence: 94%

“…They found no significant survival differences between the groups having no pulmonary valve, monocusp valves, and homograft conduits. 25 Total corrective surgery, including reconstruction of the pulmonary valve with a homograft or a heterograft, may be appropriate by the end of infancy. McCaughan et al 8 reported clinical improvement and symptom reduction in patients with APVS and TOF with a single pulmonary artery after the creation of a conduit via surgery.…”

Section: Discussionmentioning

confidence: 99%

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Absence of Left Pulmonary Artery in Tetralogy of Fallot with Absent Pulmonary Valve Syndrome

Avsar

Demir²,

Demir

2014

J Card Surg

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Section: Discussionmentioning

confidence: 94%

Section: Discussionmentioning

confidence: 99%

Absence of Left Pulmonary Artery in Tetralogy of Fallot with Absent Pulmonary Valve Syndrome

Avsar

Demir²,

Demir

2014

J Card Surg

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“…This aspect of the natural history of APVS emphasises the importance of early diagnosis and intervention before the onset of severe cardiorespiratory distress. The impact of respiratory distress at the time of surgical intervention can also be seen in a large case series by Norgaard et al ,5 where preoperative ventilator dependency was the strongest negative predictor of postoperative survival.…”

Section: Discussionmentioning

confidence: 78%

Prenatal diagnosis of absent pulmonary valve confirmed by autopsy

Vivek¹,

Shetty²,

Nayak³

et al. 2013

BMJ Case Reports

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Absent pulmonary valve syndrome (APVS) is a rare congenital cardiac anomaly. This syndrome is comprised of subtotal or total absence of pulmonary valve leaflets, stenosis of the pulmonary artery orifice, aneurysmal dilation of the main pulmonary artery and ventricular septal defect. We report a case of APVS with neural tube defect detected prenatally at 22 weeks of gestation by echocardiography, and subsequently confirmed by autopsy of the still born fetus. The common presentations, means of diagnosis and variants of APVS are discussed in brief.

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“…If children with absent pulmonary valve have early surgery with correction of the cardiac defects and pulmonary angioplasty or translocation, the prognosis is good [17, 23]. …”

Section: Discussionmentioning

confidence: 99%

Multi-slice computed tomography assessment of bronchial compression with absent pulmonary valve

Jaffe

Liu

et al. 2014

Pediatr Radiol

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BackgroundAbsent pulmonary valve is a rare cardiovascular anomaly that can result in profound tracheobronchial compression.ObjectiveTo demonstrate the advantage of multi-slice CT in diagnosing tracheobronchial compression, its severity as related to the adjacent dilated pulmonary arteries, and associated lung and cardiac lesions.Materials and methodsWe included children with absent pulmonary valve who were reviewed by multi-slice CT during a 17-year period. The number and locations of stenoses and lung lesions were noted and the severity of stenosis was categorized. The diameter of the pulmonary artery was measured and associated cardiac defects were demonstrated.ResultsThirty-one children (14 girls and 17 boys) were included. Of these, 29 had ventricular septal defect and 2 had an intact ventricular septum. Twenty-nine children (94%) had tracheobronchial compression, judged to be mild in nine children (31%), moderate in 10 (34%) and severe in 10 (34%). The different locations of the stenosis (carina, main bronchi, lobar and segmental bronchi) were observed. And the number and location of lung lesions demonstrated that the right middle and left upper and lower lobes were often affected. The diameter of the pulmonary artery in these children was well above normal published values, and Spearman rank correlation analysis showed a correlation between the size of the pulmonary artery and the severity of the tracheobronchial stenosis. Nineteen children (61%) underwent surgery and 4 of these children had a multi-slice CT post-operative follow-up study.ConclusionAbsent pulmonary valve can cause significant morbidity and mortality in children. Multi-slice CT can accurately depict areas of tracheobronchial compression, associated lung lesions and cardiac defects, helping to direct the surgeon.

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Absent pulmonary valve syndrome.☆Surgical and clinical outcome with long-term follow-up

Abstract: The surgical outcome of APVS was closely related to preoperative ventilator dependency. Efforts to improve the surgical outcome should be focused on identifying and correcting this.

Cited by 40 publications

References 17 publications

Absence of Left Pulmonary Artery in Tetralogy of Fallot with Absent Pulmonary Valve Syndrome

Absence of Left Pulmonary Artery in Tetralogy of Fallot with Absent Pulmonary Valve Syndrome

Prenatal diagnosis of absent pulmonary valve confirmed by autopsy

Multi-slice computed tomography assessment of bronchial compression with absent pulmonary valve

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