Long-Term Outcome in Patients Undergoing Surgical Repair of Tetralogy of Fallot

Murphy, Joseph G.; Gersh, Bernard J.; Mair, Douglas D.; Fuster, Valentín; McGoon, Michael D.; Ilstrup, Duane M.; McGoon, Dwight C.; Kirklin, John W.; Danielson, Gordon K.

doi:10.1056/nejm199308263290901

Cited by 887 publications

(494 citation statements)

References 22 publications

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“…Fallot tetralojisinin tedavisi cerrahidir. Tam düzelt-me yapılan TOF'lu hastaların %85'inin erişkin çağa ulaşabildikle-ri gözlemlenmektedir (2). Eskiden tam düzeltme için iki yaşına kadar beklemek tercih edilirken, son 30 yıllık süreçte eğer pulmoner yatak uygunsa, bir yaşından önce tam düzetme yapılmak-tadır.…”

Section: Introductionunclassified

Determinants of short-term mortality and morbidity after the complete repair of tetralogy of Fallot in infant groups under 12 months and one-four years of age

Şaşmazel¹,

Fedakar²,

Baysal³

et al. 2010

Anadolu Kardiyol Derg

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Fallot tetralojili on iki ay altı infantlar ile bir-dört yaş arası çocuk hasta gruplarında tam düzeltme ameliyatı sonrasında kısa dönem mortalite ve morbiditenin belirleyicileriDeterminants of short-term mortality and morbidity after the complete repair of tetralogy of Fallot in infant groups under 12 months and one-four years of age Özgün Araşt›rma Original Investigation 544Yaz›şma Adresi/Address ABSTRACTObjective: In patients with tetralogy of Fallot, infants less than 12 months old and children between one and four years old were compared after total repair surgery for determination of outcome of surgery, risk factors influencing mortality and morbidity. Methods: Fifty-two patients with tetralogy of Fallot between five months and four years of ages were included into the study. The patients were divided into two groups depending on their ages; Group 1; one year old and younger (n=21) whereas, group 2; one and four years old (n=31). Statistical analysis was performed using Mann-Whitney U, Chi-square or where appropriate Fisher's exact tests and logistic regression analysis was applied for determination of predictors of mortality.Results: The parameters that were different between two groups include; age (Group 1; 10.00±1.67 months and Group 2; 2.39±0.77 years, p<0.001), weight (Group 1; 9.74±2.23 kg, Group 2; 11.97±1.78 kg, p<0.001), McGoon ratio (Group 1; 1.94±0.29, Group 2; 2.19±0.27, p=0.001). Mortality is found in 3 patients in group 1 (14.2%) whereas, in five patients in group 2 (16%) and the difference was not statistically significant. In group 2 in only one patient (0.03%) had complete atrioventricular block and required permanent pacemaker implantation. When patients were compared according to groups with and without mortality, the significant differences were found in following variables: peritoneal dialysis (p=0.001), pleural effusion (p=0.02), right ventricular pressure (p=0.001) and right ventricle/aorta pressures ratio (p=0.001). However, none of these risk factors had significant value in prediction of mortality. GirişFallot tetralojisi (TOF) tüm doğumsal kalp hastalıklarının %10'nu oluşturur ve siyanotik kalp hastalıklarının da en başta gelenidir (1). Fallot tetralojisinin tedavisi cerrahidir. Tam düzelt-me yapılan TOF'lu hastaların %85'inin erişkin çağa ulaşabildikle-ri gözlemlenmektedir (2). Eskiden tam düzeltme için iki yaşına kadar beklemek tercih edilirken, son 30 yıllık süreçte eğer pulmoner yatak uygunsa, bir yaşından önce tam düzetme yapılmak-tadır. Erken dönemde tam düzeltme yapılması, iki aşamalı ameliyatın getirdiği riski ortadan kaldırır. Pulmoner arterlere ve akciğer parankimine erken dönemde normal kan akımını sağlayarak, kronik hipokseminin etkilerinden korur. Sağ ventrikül önündeki basınç yükü kalktığından sağ ventrikül hipertrofisi geriler. BarattBoyes (3) 1969'da, Castañeda ve ark. (4) 1972'de erken dönemde tam düzeltme ameliyatı sonrası iyi sonuçlarını bildirmişlerdir (5). Bu çalışmada TOF'lu 12 ay altı infantlar ile bir-dört yaş arası çocuk hasta gruplarında tam düzeltme am...

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Section: Introductionunclassified

Determinants of short-term mortality and morbidity after the complete repair of tetralogy of Fallot in infant groups under 12 months and one-four years of age

Şaşmazel¹,

Fedakar²,

Baysal³

et al. 2010

Anadolu Kardiyol Derg

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“…[1] Pulmonary valve (PV) regurgitation resulting from surgical repair, particularly after incision of the PV annulus and transannular patch for repair of tetralogy of Fallot or other right ventricular outflow tract obstruction with hypoplastic PV annulus, is generally quite well tolerated in the post-operative period, and it was initially thought to be a quite benign condition. [2] However, on longer-term follow-up and subsequent review, the presence of severe PV regurgitation was reported to be associated with exercise intolerance, heart failure, ventricular arrhythmias with syncopal episodes and sudden death.…”

Section: Introductionmentioning

confidence: 99%

Trifecta St. Jude medical® aortic valve in pulmonary position

Corno

Dawson

Bolger

et al. 2017

Nano Reviews & Experiments

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Introduction: To evaluate an aortic pericardial valve for pulmonary valve (PV) regurgitation after repair of congenital heart defects. Methods: From July 2012 to June 2016 71 patients, mean age 24 ± 13 years (four to years) underwent PV implantation of aortic pericardial valve, mean interval after previous repair = 21 ± 10 years (two to 47 years). Previous surgery at mean age 3.2 ± 7.2 years (one day to 49 years): tetralogy of Fallot repair in 83% (59/71), pulmonary valvotomy in 11% (8/71), relief of right ventricular outflow tract (RVOT) obstruction in 6% (4/71). Pre-operative echocardiography and MRI showed severe PV regurgitation in 97% (69/71), moderate in 3% (2/71) with associated RVOT obstruction. MRI and knowledge-based reconstruction 3D volumetry (KBR-3D-volumetry) showed mean PV regurgitation = 42 ± 9% (20–58%), mean indexed RV end-diastolic volume = 169 ± 33 (130–265) ml m–2 BSA and mean ejection fraction (EF) = 46 ± 8% (33–61%). Cardio-pulmonary exercise showed mean peak O2/uptake = 24 ± 8 ml kg–1 min–1 (14–45 ml kg–1 min–1), predicted max O2/uptake 66 ± 17% (26–97%). Pre-operative NYHA class was I in 17% (12/71) patients, II in 70% (50/71) and III in 13% (9/71). Results: Mean cardio-pulmonary bypass duration was 95 ± 30ʹ (38–190ʹ), mean aortic cross-clamp in 23% (16/71) 46 ± 31ʹ (8–95ʹ), with 77% (55/71) implantations without aortic cross-clamp. Size of implanted PV: 21 mm in seven patients, 23 mm in 33, 25 mm in 23, and 27 mm in eight. The z-score of the implanted PV was −0.16 ± 0.80 (−1.6 to 2.5), effective orifice area indexed (for BSA) of native PV was 1.5 ± 0.2 (1.2 to –2.1) vs. implanted PV 1.2 ± 0.3 (0.76 to –2.5) (p = ns). In 76% (54/71) patients surgical RV modelling was associated. Mean duration of mechanical ventilation was 6 ± 5 h (0–26 h), mean ICU stay 21 ± 11 h (12–64 h), mean hospital stay 6 ± 3 days (three to 19 days). In mean follow-up = 25 ± 14 months (six to 53 months) there were no early/late deaths, no need for cardiac intervention/re-operation, no valve-related complications, thrombosis or endocarditis. Last echocardiography showed absent PV regurgitation in 87.3% (62/71) patients, trivial/mild degree in 11.3% (8/71), moderate degree in 1.45% (1/71), mean max peak velocity through RVOT 1.6 ± 0.4 (1.0–2.4) m s–1. Mean indexed RV end-diastolic volume at MRI/KBR-3D-volumetry was 96 ± 20 (63–151) ml m–2 BSA, lower than pre-operatively (p < 0.001), and mean EF = 55 ± 4% (49–61%), higher than pre-operatively (p < 0.05). Almost all patients (99% = 70/71) remain in NYHA class I, 1.45% = 1/71 in class II. Conclusion: (a) Aortic pericardial valve is implantable in PV position with an easy and reproducible surgical technique; (b) valve size adequate for patient BSA can be implanted with simultaneous RV remodelling; (c) medium-term outcomes are good with maintained PV function, RV dimensions significantly reduced and EF significantly improved; (d) adequate valve size will allow later percutaneous valve-in-valve implantation.

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“…Tetralogy of Fallot (TOF) is the most common cyanotic congenital heart disease (CHD) [1]. Albeit TOF is defined by changes of the right ventricle and the pulmonary vessels, also dilatation of the aortic root and the ascending aorta can occur [2].…”

Section: Introductionmentioning

confidence: 99%

Dimensions of the Ascending Aorta in Children and Adolescents with Repaired Tetralogy of Fallot Obtained by Cardiac Magnetic Resonance Angiography

Mende¹,

Gräfe²,

Dähnert³

et al. 2016

Thorac cardiovasc Surg

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Introduction Dilatation of the ascending aorta is a common finding in Tetralogy of fallot (TOF). We sought to provide aortic dimensions in children and adolescents after corrected TOF obtained by contrast-enhanced cardiacmagnetic-resonance angiography (CE-CMRA) that could serve as reference values. Materials and methods We enrolled 101 children and adolescents (56 male) with a median age of 10.9 years. All patients underwent CE-CMRA imaging using a 3-dimensional spoiled gradient-echo-sequence. Aortic diameters were measured at the level of the aortic valve (AV), aortic sinus (AS), sino-tubular junction (STJ) and the ascending aorta (AA) and compared with normal values obtained from literature. Sex-specific aortic dimensions are given as percentile curves as well as z scores. Furthermore CMR volumetric and functional parameters as well as clinical and anamnestic data were analyzed to identify parameters that are associated with aortic dilatation. Results Diameters for aortic size for males were 3.6 ? 16.6*BSA 0.5 at the AV level, 7.0 ? 19.5*BSA 0.5 at the AS level, 7.0 ? 14.4*BSA 0.5 at the STJ level and 7.3 ? 15.5*BSA 0.5 at the AA level. Diameters for females were 5.8 ? 14.1*BSA 0.5 at the AV level, 7.2 ? 17.6*BSA 0.5 at the AS level, 5.2 ? 15.4*BSA 0.5 at the STJ level and 2.0 ? 17.8*BSA 0.5 at the AA level. All diameters in TOF patients were larger compared with normal values. The postoperative interval and age at examination were the only parameters associated with aortic size at all measured levels. Conclusion We provide CE-CMRA data of aortic dimensions in children and adolescents after correction of TOF. Our data might be useful for an estimation of the ''normal'' aortic size in this patient cohort and can serve as a basis for future longitudinal studies adding prognostic data.

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Long-Term Outcome in Patients Undergoing Surgical Repair of Tetralogy of Fallot

Abstract: Among patients with surgically repaired tetralogy of Fallot, the rate of long-term survival after the postoperative period is excellent but remains lower than that in the general population. The risk of late sudden death is small.

Cited by 887 publications

References 22 publications

Determinants of short-term mortality and morbidity after the complete repair of tetralogy of Fallot in infant groups under 12 months and one-four years of age

Determinants of short-term mortality and morbidity after the complete repair of tetralogy of Fallot in infant groups under 12 months and one-four years of age

Trifecta St. Jude medical® aortic valve in pulmonary position

Dimensions of the Ascending Aorta in Children and Adolescents with Repaired Tetralogy of Fallot Obtained by Cardiac Magnetic Resonance Angiography

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