Pulmonary vein stenosis with normal connection: associated cardiac abnormalities and variable outcome

Breinholt, John P.; Hawkins, John A.; Minich, L. Lu Ann; Tani, Lloyd Y.; Orsmond, Garth S.; Ritter, Saskia; Shaddy, Robert E.

doi:10.1016/s0003-4975(99)00311-2

Cited by 90 publications

(78 citation statements)

References 11 publications

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Section: Discussionmentioning

confidence: 99%

“…2,[18][19][20] Although affecting a small number of patients, recurrent post repair PV stenosis may be lethal if it develops bilaterally. 1,3,6,7,20 In patients with right isomerism, the presence of an obstructed PV is an independent risk factor for a poor prognosis. 21 A previous study suggested that the presence of echocardiographically defined turbulence within the PVs after repair is associated with restenosis, 22 and another study reported that the size of the individual PVs was an important determinant of outcome in patients with TAPVC.…”

Section: Discussionmentioning

confidence: 99%

See 1 more Smart Citation

Stent Implantation and Subsequent Dilatation for Pulmonary Vein Stenosis in Pediatric Patients. Maximizing Effectiveness.

Tomita

Watanabe

Yazaki

et al. 2003

Circ J

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Stent Implantation and Subsequent Dilatation for Pulmonary Vein Stenosis in Pediatric Patients. Maximizing Effectiveness.

Tomita

Watanabe

Yazaki

et al. 2003

Circ J

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“…Previous studies have shown that pulmonary vein stenosis is a progressive disease. 9,[25][26][27] We recently showed that a discrete obstruction of the pulmonary veins leads to pulmonary venous remodeling, small pulmonary veins, and even development of pulmonary vein atresia. 28 TAPVC may be obstructed in utero.…”

Section: Overall Survival and Risk Factors For Deathmentioning

confidence: 99%

Total Anomalous Pulmonary Venous Connection

Seale

Uemura²,

Webber³

et al. 2010

Circulation

236

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on behalf of the British Congenital Cardiac AssociationBackground-Late mortality after repair of total anomalous pulmonary venous connection is frequently associated with pulmonary venous obstruction (PVO). We aimed to describe the morphological spectrum of total anomalous pulmonary venous connection and identify risk factors for death and postoperative PVO. Methods and Results-We conducted a retrospective, international, collaborative, population-based study involving all 19 pediatric cardiac centers in the United Kingdom, Ireland, and Sweden. All infants with total anomalous pulmonary venous connection born between 1998 and 2004 were identified. Cases with functionally univentricular circulations or atrial isomerism were excluded. All available data and imaging were reviewed. Of 422 live-born cases, 205 (48.6%) had supracardiac, 110 (26.1%) had infracardiac, 67 (15.9%) had cardiac, and 37 (8.8%) had mixed connections. There were 2 cases (0.5%) of common pulmonary vein atresia. Some patients had extremely hypoplastic veins or, rarely, discrete stenosis of the individual veins. Sixty (14.2%) had associated cardiac anomalies. Sixteen died before intervention. Three-year survival for surgically treated patients was 85.2% (95% confidence interval 81.3% to 88.4%). Risk factors for death in multivariable analysis comprised earlier age at surgery, hypoplastic/stenotic pulmonary veins, associated complex cardiac lesions, postoperative pulmonary hypertension, and postoperative PVO. Sixty (14.8%) of the 406 patients undergoing total anomalous pulmonary venous connection repair had postoperative PVO that required reintervention. Three-year survival after initial surgery for patients with postoperative PVO was 58.7% (95% confidence interval 46.2% to 69.2%). Risk factors for postoperative PVO comprised preoperative hypoplastic/stenotic pulmonary veins and absence of a common confluence. Key Words: follow-up studies Ⅲ pulmonary veins Ⅲ pulmonary vein stenosis Ⅲ heart defects, congenital T otal anomalous pulmonary venous connection (TAPVC) is a rare form of congenital heart disease in which all pulmonary veins connect to the systemic veins, right atrium, or coronary sinus. TAPVC can occur in conjunction with a wide variety of cardiac malformations, especially atrial isomerism. Before the advent of cardiac surgery, almost all of these children died in the first few months of life. With treatment, there has been continued improvement in the mortality and morbidity of isolated TAPVC. [1][2][3][4] This can be attributed to progress in surgical expertise and to developments in intensive care such as use of nitric oxide and extracorporeal membrane oxygenation, which have led to salvage of the sickest neonates. Conclusions-Preoperative Clinical Perspective on p 2726Nevertheless, there is an ongoing late mortality in patients with TAPVC. It is frequently associated with postoperative pulmonary venous obstruction (PVO), with an incidence of 5% to 18%. [1][2][3][5][6][7][8][9] Postoperative PVO may be a consequence of an inadequate ana...

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“…The finding of turbulent flow on echocardiography with Doppler ultrasound should raise the suspicion of PVS (2). Because patients with PVS often have cardiac anomalies, pulmonary hypertension may mistakenly be attributed to the cardiac findings instead of the underlying PVS (2,9). In addition to the right ventricular failure in the present case, our patient also had mild BPD that was out of proportion to the degree of pulmonary hypertension.…”

Section: Discussionmentioning

confidence: 54%

“…Noninvasive tests such as echocardiography with Doppler ultrasound, magnetic resonance imaging, multidetector CT angiography and radionuclide quantitative pulmonary flow imaging may detect PVS (2,9). The finding of turbulent flow on echocardiography with Doppler ultrasound should raise the suspicion of PVS (2).…”

Section: Discussionmentioning

confidence: 99%

Pulmonary Vein Stenosis: Case Report and Literature Review

Amin

Kwon

Moayedi

et al. 2009

Canadian Respiratory Journal

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BACKGROUND: Pulmonary vein stenosis is a rare cause of pulmonary hypertension, with variable onset and presentation. One or more of the four pulmonary veins can be primarily or secondarily affected. A five-month-old girl presented with respiratory distress, lethargy and cyanosis requiring intubation.METHODS: Echocardiography showed right ventricular dilation, a right ventricular systolic pressure of 97 mmHg, decreased ejection fraction and turbulent flow at the left atrium. Cardiac catheterization revealed stenosis of the left-sided pulmonary veins, for which she underwent a Coles procedure.RESULTS: Postoperatively, there was a transient improvement in the patient’s pulmonary hypertension but she subsequently deteriorated. Her prognosis was considered bleak, and a decision was made with the family to withdraw care.CONCLUSION: Pulmonary vein stenosis is a rare cause of pulmonary hypertension, and is associated with significant morbidity and mortality. Surgical intervention may be of benefit in selected cases. It stands to reason that any treatment will have the best chance of success if completed before the pulmonary hypertension becomes fixed. Early diagnosis depends on a high index of clinical suspicion.

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Pulmonary vein stenosis with normal connection: associated cardiac abnormalities and variable outcome

Cited by 90 publications

References 11 publications

Stent Implantation and Subsequent Dilatation for Pulmonary Vein Stenosis in Pediatric Patients. Maximizing Effectiveness.

Stent Implantation and Subsequent Dilatation for Pulmonary Vein Stenosis in Pediatric Patients. Maximizing Effectiveness.

Total Anomalous Pulmonary Venous Connection

Pulmonary Vein Stenosis: Case Report and Literature Review

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