Natural and unnatural history of pulmonary atresia

Leonard, H; Derrick, Graham; O’Sullivan, John; Wren, Christopher

doi:10.1136/heart.84.5.499

Cited by 108 publications

(86 citation statements)

References 22 publications

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“…Data addressing the growth of RV structures (tricuspid valve, chamber dimensions) in patients achieving a biventricular circulation using this strategy have not been analyzed previously. Surgical repair for the neonate with PA-IVS is associated with a high morbidity, a 1-year mortality of 52%, 9 and a 4-year survival of 64% from a recent surgical multicenter study. 10 In a highly selective patient population, 98% survival can be achieved.…”

Section: Discussionmentioning

confidence: 99%

Percutaneous Balloon Valvotomy in Pulmonary Atresia With Intact Ventricular Septum

et al. 2003

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Background— Pulmonary atresia with intact ventricular septum (PA-IVS) is a rare congenital lesion with high mortality. Therapy was exclusively surgical until recently, when the use of radiofrequency-assisted perforation of the atretic valve was introduced as a treatment option. This study analyzes the outcomes and morphological changes to right heart structures after percutaneous perforation and balloon dilation of the atretic valve. Methods and Results— Between April 1992 and August 2000, 30 patients with PA-IVS underwent attempted percutaneous valve perforation and balloon dilation of the pulmonary valve. Longitudinal echocardiographic measurements of the tricuspid valve diameter, right ventricular length and area were recorded. Z scores were calculated according to published formulas. Perforation was achieved in 27 patients. In 14 patients a modified Blalock-Taussig shunt was performed between 2 and 24 days after valve dilation. There were 3 early and 2 late deaths. Among the survivors (follow-up time of 1 to 87 months), 16 patients had a biventricular circulation, 3 a 1 ½-ventricle circulation, and 1 a Fontan operation. Four patients are awaiting further palliation. There was no significant change of the tricuspid valve Z score or right ventricular length Z score with time. Conclusions— Percutaneous balloon valvotomy is an effective treatment strategy for patients with PA-IVS provided that there is a patent infundibulum and a lack of a right ventricle–dependent coronary circulation. Despite the observation that right heart growth does not increase with body growth in early follow-up, it appears adequate to maintain a biventricular circulation in many patients.

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Section: Discussionmentioning

confidence: 99%

Percutaneous Balloon Valvotomy in Pulmonary Atresia With Intact Ventricular Septum

et al. 2003

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“…Leonard and colleagues 13 report a total mortality of 56% with one-fifth of deaths occurring in the first week and two-thirds within the first year (for infants live-born in 1980-95). One-year mortality was 52% for PA/IVS, 25% for PA/VSD and 48% for PA associated with more complex abnormalities.…”

Section: Outcomementioning

confidence: 99%

“…In Hoffman and Kaplan's review of 11 papers, a median prevalence of 8 per 100,000 but a mean of 13 per 100,000 is cited (lower quartile 8, upper quartile 15). 11 In the North East of England, the prevalence at live birth of all forms of pulmonary atresia was 21 per 100,000, with 5 per 100,000 with PA/IVS, 10 per 100,000 with PA/VSD and 7 per 100,000 with more complex PA. 13 However, the reported prevalence depends on the hierarchy used to describe infants with more than one cardiac malformation. An 'anatomical' or 'embryological' hierarchy will lead to under reporting of PA by 27% when compared with a 'physiological' hierarchy.…”

Section: Prevalence At Live Birthmentioning

confidence: 99%

Newborn screening for congenital heart defects: a systematic review and cost-effectiveness analysis

Knowles¹,

Griebsch²,

Dezateux

et al. 2005

Health Technol Assess

212

143

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Non-UK purchasers will have to pay a small fee for post and packing. For European countries the cost is £2 per monograph and for the rest of the world £3 per monograph.You can order HTA monographs from our Despatch Agents:-fax (with credit card or official purchase order) -post (with credit card or official purchase order or cheque) -phone during office hours (credit card only).Additionally the HTA website allows you either to pay securely by credit card or to print out your order and then post or fax it. NHS libraries can subscribe free of charge. Public libraries can subscribe at a very reduced cost of £100 for each volume (normally comprising 30-40 titles). The commercial subscription rate is £300 per volume. Please see our website for details. Subscriptions can only be purchased for the current or forthcoming volume. Contact details are as follows: Payment methods Paying by chequeIf you pay by cheque, the cheque must be in pounds sterling, made payable to Direct Mail Works Ltd and drawn on a bank with a UK address. Paying by credit cardThe following cards are accepted by phone, fax, post or via the website ordering pages: Delta, Eurocard, Mastercard, Solo, Switch and Visa. We advise against sending credit card details in a plain email. Paying by official purchase orderYou can post or fax these, but they must be from public bodies (i.e. NHS or universities) within the UK. We cannot at present accept purchase orders from commercial companies or from outside the UK. How do I get a copy of HTA on CD?Please use the form on the HTA website (www.hta.ac.uk/htacd.htm). Or contact Direct Mail Works (see contact details above) by email, post, fax or phone. HTA on CD is currently free of charge worldwide.The website also provides information about the HTA Programme and lists the membership of the various committees. HTANewborn screening for congenital heart defects: a systematic review and cost-effectiveness analysis NHS R&D HTA ProgrammeT he research findings from the NHS R&D Health Technology Assessment (HTA) Programme directly influence key decision-making bodies such as the National Institute for Health and Clinical Excellence (NICE) and the National Screening Committee (NSC) who rely on HTA outputs to help raise standards of care. HTA findings also help to improve the quality of the service in the NHS indirectly in that they form a key component of the 'National Knowledge Service' that is being developed to improve the evidence of clinical practice throughout the NHS.The HTA Programme was set up in 1993. Its role is to ensure that high-quality research information on the costs, effectiveness and broader impact of health technologies is produced in the most efficient way for those who use, manage and provide care in the NHS. 'Health technologies' are broadly defined to include all interventions used to promote health, prevent and treat disease, and improve rehabilitation and long-term care, rather than settings of care.The HTA Programme commissions research only on topics where it has identified key gaps in the evi...

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“…15) TOF with MAPCAs has a clinical course that is frequently complicated by the development of PAH. The MAPCAs that supply blood to the lungs vary in terms of their size, location, and blood flow volume between patients.…”

Section: Discussionmentioning

confidence: 99%

Pulmonary Arterial Hypertension Associated With Tetralogy of Fallot

Yasuhara

Yamagishi

2015

Int. Heart J.

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SummaryPulmonary arterial hypertension (PAH) is a common postoperative complication in patients with congenital heart disease (CHD). Although the recent clinical classification of pulmonary hypertension divided PAH associated with CHD (PAH-CHD) into several subclasses, the anatomical and hemodynamic features of postoperative PAH-CHD vary enormously. Therefore, it is still difficult to obtain clinical evidence supporting the indication of pulmonary vasodilators for PAH-CHD. We often encounter patients with PAH occurring after surgical treatment of tetralogy of Fallot (TOF), especially patients with major aortopulmonary collateral arteries (MAPCAs). PAH might be caused by pulmonary agenesis, hypoplasia and/or thrombosis, inadequate closure of the ventricular septal defect, relief of the pulmonic stenosis, or an excessively large prior systemic-to-pulmonary shunt. Moreover, patients with TOF and MAPCAs who are diagnosed as inoperable because of the presence of PAH show similar hemodynamic and clinical features to patients with Eisenmenger syndrome. The MAPCAs in these patients usually show hypoplastic and abnormal arborization. Based on our experience, we believe that PAH-targeted therapies are effective in some patients with PAH occurring after surgical treatment of TOF and MAPCAs, especially as an adjunct to percutaneous pulmonary angioplasty. To help classify patients with PAH associated with TOF, especially with MAPCAs, we propose several new subclassifications: "PAH due to hypoplastic pulmonary arterial beds", "PAH due to abnormal pulmonary arborization", or "segmental PAH associated with CHD." A multicenter registry of patients using a unified protocol is essential to explore the indications and efficacy of pulmonary vasodilators for postoperative PAH-CHD. (Int Heart J 2015; 56: S17-S21) Key words: Major aortopulmonary collateral artery, Pulmonary vasodilator, Percutaneous pulmonary angioplasty, Pulmonary arterial bed P ulmonary arterial hypertension (PAH) is a frequent complication in patients with congenital heart disease (CHD) and has significant effects on their prognosis. In patients with systemic-to-pulmonary shunts, prolonged increases in blood flow and pressure in the pulmonary arteries promotes vascular remodeling and dysfunction, leading to increased pulmonary vascular resistance. Further progression of this condition ultimately results in shunt reversal and the development of Eisenmenger syndrome, one of the most advanced types of PAH associated with CHD (PAH-CHD), as discussed in another chapter of this issue.In recent years, the prevalence of PAH-CHD has decreased in developed countries and the number of patients surviving into adulthood has increased. The timing of corrective surgery of CHD is critical to avoid pulmonary vascular remodeling and PAH.1) Because the majority of patients with a systemic-to-pulmonary shunt undergo heart surgery at an appropriate time, the prevalence of Eisenmenger syndrome has decreased substantially in developed countries. However, 4-15% of patients with CHD still d...

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Natural and unnatural history of pulmonary atresia

Abstract: Objective-To investigate mortality, cause of death, survival, and quality of life in all types of cardiac malformation with congenital pulmonary atresia. Design-Retrospective analysis.

Cited by 108 publications

References 22 publications

Percutaneous Balloon Valvotomy in Pulmonary Atresia With Intact Ventricular Septum

Percutaneous Balloon Valvotomy in Pulmonary Atresia With Intact Ventricular Septum

Newborn screening for congenital heart defects: a systematic review and cost-effectiveness analysis

Pulmonary Arterial Hypertension Associated With Tetralogy of Fallot

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