Early and Late Results of the Modified Fontan Operation for Heterotaxy Syndrome

Bartz, Peter J.; Driscoll, David J.; Dearani, Joseph A.; Puga, Francisco J.; Danielson, Gordon K.; O’Leary, Patrick; Earing, Michael G.; Warnes, Carole A.; Hodge, David O.; Cetta, Frank

doi:10.1016/j.jacc.2006.07.053

Cited by 146 publications

(125 citation statements)

References 15 publications

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“…Death was considered perioperative if it occurred within 30 days of surgery or before hospital discharge 19 and was subclassified as early (ie, within 30 days) or late (ie, after 30 days but during the same hospitalization). Death was labeled secondary to heart failure if it complicated worsening heart failure, as defined by evidence of at least one of the following: orthopnea, nocturnal dyspnea, pulmonary edema, increasing peripheral edema, renal hypoperfusion (ie, worsening renal function), or radiological signs of congestive heart failure.…”

Section: Mode Of Deathmentioning

confidence: 99%

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

Khairy¹,

Fernandes²,

Mayer³

et al. 2008

Circulation

880

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: Mode Of Deathmentioning

confidence: 99%

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

Khairy¹,

Fernandes²,

Mayer³

et al. 2008

Circulation

880

622

View full text Add to dashboard Cite

show abstract

“…Death was considered perioperative if it occurred within 30 days of surgery or before hospital discharge and was subclassified as early (ie, within 30 days) or late (ie, after 30 days but during the same hospitalization). 23 Death was classified as secondary to heart failure if it complicated worsening heart failure, as defined by evidence of ≥1 of the following: orthopnea, nocturnal dyspnea, pulmonary edema, increasing peripheral edema, renal hypoperfusion (ie, worsening renal function), or radiological signs of congestive heart failure. 24 Sudden death was defined as death occurring within 1 hour of acute symptoms.…”

Section: Mode Of Deathmentioning

confidence: 99%

Cardiovascular Outcomes After the Arterial Switch Operation for D-Transposition of the Great Arteries

et al. 2013

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Background— Data regarding long-term outcomes after the arterial switch operation for D-transposition of the great arteries are scarce. Methods and Results— A single-institution retrospective cohort study was conducted to assess cardiovascular outcomes after an arterial switch operation between 1983 and 1999. Patients without follow-up visits within 3 years were contacted and secondary sources of information obtained. Overall, 400 patients, 154 (38.3%) with a ventricular septal defect, 238 (59.5%) with an intact septum, and 9 (2.3%) with a Taussig-Bing anomaly, were followed for a median of 18.7 years. In perioperative survivors, overall and arrhythmia-free survival rates at 25 years were 96.7±1.8% and 96.6±0.1%, respectively. Late mortality was predominantly a result of sudden deaths and myocardial infarction. At 25 years, 75.5±2.5% remained free from surgical or catheter-based reintervention. Freedom from an adverse cardiovascular event was 92.9±1.9% at 25 years. Independent predictors were a single right coronary artery (hazard ratio, 4.58; 95% confidence interval, 1.32-15.90), P =0.0166) and postoperative heart failure (hazard ratio, 6.93; 95% confidence interval, 1.57-30.62; P =0.0107). At last follow-up, the left ventricular ejection fraction was 60.3±8.9%, 97.3% had class I symptoms, and 5.2% obstructive coronary artery disease. Peak oxygen uptake was 35.1±7.6 mL/kg/min (86.1±15.1% predicted), with a chronotropic index <80% in 34.2%. At least moderate neoaortic and pulmonary regurgitation were present in 3.4% and 6.6%, respectively, and more than mild neoaortic and pulmonary stenosis in 3.2% and 10.3%. Conclusions— Long-term and arrhythmia-free survival is excellent after arterial switch operation. Although sequelae include chronotropic incompetence and neoaortic, pulmonary, and coronary artery complications, most patients maintain normal systolic function and exercise capacity.

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“…13, 14 The mid-to long-term prognosis of heterotaxy patients who undergo the Fontan procedure for single ventricular physiology is now an important issue in pediatric and adult CHD clinics. [15][16][17][18] Human Heterotaxy Syndrome sary to understand the underlying molecular and cellular mechanisms of determination of the left-right body axis during early embryogenesis. [8][9][10][11][12] This process initiates in the primitive node at E7.5 in mice and develops through the following pathways: 19,20 (1) breaking of symmetry as a result of the leftward nodal flow generated by rotational movement of primary cilia in the node; (2) transmission of asymmetric signals to the LPM; (3) asymmetric expression of Nodal and Lefty2, which encodes a feedback inhibitor of Nodal signaling, in the LPM; and (4) situs-specific morphogenesis mediated by asymmetric expression of Pitx2, which encodes a transcription factor and is regulated by Nodal signaling.…”

mentioning

confidence: 99%

Human Heterotaxy Syndrome

Shiraishi

Ichikawa

2012

Circ J

121

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Early and Late Results of the Modified Fontan Operation for Heterotaxy Syndrome

Abstract: Early survival has improved for heterotaxy patients after the Fontan operation; however, late morbidity and mortality remain substantial. Better strategies for long-term treatment of this high-risk group need to be identified.

Cited by 146 publications

References 15 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

Cardiovascular Outcomes After the Arterial Switch Operation for D-Transposition of the Great Arteries

Human Heterotaxy Syndrome

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