Outcome and assessment after the modified Fontan procedure for hypoplastic left heart syndrome.

Farrell, Paul E.; Chang, Anthony; Murdison, Kenneth A.; Baffa, Jeanne M.; Norwood, William I.; Murphy, John D.

doi:10.1161/01.cir.85.1.116

Cited by 56 publications

(18 citation statements)

References 16 publications

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“…5,6,25 In addition to early surgical era, we and others noted higher perioperative mortality rates in hypoplastic left heart syndrome. 8,26,27 In a crude analysis, Ono et al 13 similarly found significantly higher mortality with RA-PA versus total cavopulmonary connection. Although our multivariate analyses controlled for date of surgery and Fontan fenestration, relevant surgical variables not considered include cardiopulmonary bypass time, aortic cross-clamping, and modified ultrafiltration.…”

Section: Khairy Et Al Mortality In Fontan Patients 89mentioning

confidence: 99%

Long-Term Survival, Modes of Death, and Predictors of Mortality in Patients With Fontan Surgery

Khairy¹,

Fernandes²,

Mayer³

et al. 2008

Circulation

881

622

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Background-To better define determinants of mortality in patients with univentricular physiology, a database registry was created of patients born in 1985 or earlier with Fontan surgery who were followed up at Children's Hospital Boston. Methods and Results-A total of 261 patients, 121 of whom (46.4%) were women, had a first Fontan surgery at a median age of 7.9 years: right atrium-to-pulmonary artery connection in 135 (51.7%); right atrium to right ventricle in 25 (9.6%); and total cavopulmonary connection in 101 (38.7%). Over a median of 12.2 years, 76 (29.1%) died, 5 (1.9%) had cardiac transplantation, 5 (1.9%) had Fontan revision, and 21 (8.0%) had Fontan conversion. Perioperative mortality decreased steadily over time and accounted for 68.4% of all deaths. In early survivors, actuarial freedom from death or transplantation was 93.7%, 89.9%, 87.3%, and 82.6% at 5, 10, 15, and 20 years, respectively, with no significant difference between right atrium to pulmonary artery versus total cavopulmonary connection. Late deaths were classified as sudden in 7 patients (9.2%), thromboembolic in 6 (7.9%), heart failure-related in 5 (6.7%), sepsis in 2 (2.6%), and other in 4 (5.2%). Most sudden deaths were of presumed arrhythmic origin with no identifiable predictor. Independent risk factors for thromboembolic death were lack of antiplatelet or anticoagulant therapy (hazard ratio [HR], 91.6; Pϭ0.0041) and clinically diagnosed intracardiac thrombus (HR, 22.7; Pϭ0.0002

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Section: Khairy Et Al Mortality In Fontan Patients 89mentioning

confidence: 99%

Long-Term Survival, Modes of Death, and Predictors of Mortality in Patients With Fontan Surgery

Khairy¹,

Fernandes²,

Mayer³

et al. 2008

Circulation

881

622

View full text Add to dashboard Cite

show abstract

“…Initially, the Fontan procedure was done without staged interventions. This strategy was accompanied with a high operative mortality (16-40%) [7,8].…”

Section: Infant Periodmentioning

confidence: 99%

Anesthetic management of noncardiac surgery for patients with single ventricle physiology

2011

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Patients with congenital heart diseases are a growing population, and noncardiac surgeries will become an important health care issue. Patients with single ventricle physiology are a particularly challenging population who will undergo staged, palliative repair toward a final step of Fontan circulation. Although Fontan surgery creates a serial circulation in which the ventricle pumps blood to the systemic circuit, pulmonary blood flow occurs without a dedicated ventricle. Despite progress in outcomes, this abnormal circulation remains associated with various co-morbidities such as ventricular dysfunction, arrhythmias, protein losing enteropathy, and plastic bronchitis. Health care professionals must prepare for these patients to present to noncardiac surgery at any stage of intervention, possibly with complications. Given that staged, palliative repair has undergone multiple modifications, patients who present for surgery can vary in types and timing of the repair. Anesthesiologists who care for them must be familiar with perioperative issues to optimize outcomes, especially because congenital heart disease is a risk factor for increased mortality for noncardiac surgery.

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“…Intermediate survival after modified Fontan surgery in patients with hypoplastic left heart syndrome appears comparable to other complex functionally single ventricle patients [3]. Subjects who have had a Fontan operation for a functionally single ventricle have reduced exercise performance as compared with normal controls [2].…”

mentioning

confidence: 91%

Exercise Performance Following Repair of Hypoplastic Left Heart Syndrome: A Comparison with Other Types of Fontan Patients

et al. 1997

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Reports of exercise performance after Fontan surgery for hypoplastic left heart syndrome (HLHS) are lacking. We compared the exercise performance of total cavopulmonary connection type (TCPC) of Fontan subjects with HLHS (group 1, n = 7) to those not requiring a Norwood procedure having a systemic right ventricle (group 2, n = 6) or a systemic left ventricle (group 3, n = 8). The subjects underwent assessment of resting pulmonary mechanics followed by maximal exercise testing with a bicycle or treadmill protocol. ECG, oxygen consumption, and carbon dioxide production were measured continuously. There was not a significant difference seen between HLHS and the comparison groups for the following parameters: maximum heart rate, maximum oxygen consumption, respiratory exchange ratio, breathing reserve, and arterial oxygen saturation at rest or exercise. Exercise performance in the TCPC type of Fontan patients was comparable regardless of ventricular morphology or surgical approach.

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Outcome and assessment after the modified Fontan procedure for hypoplastic left heart syndrome.

Cited by 56 publications

References 16 publications

Long-Term Survival, Modes of Death, and Predictors of Mortality in Patients With Fontan Surgery

Long-Term Survival, Modes of Death, and Predictors of Mortality in Patients With Fontan Surgery

Anesthetic management of noncardiac surgery for patients with single ventricle physiology

Exercise Performance Following Repair of Hypoplastic Left Heart Syndrome: A Comparison with Other Types of Fontan Patients

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