Timing and type of surgery for severe pulmonary regurgitation after repair of tetralogy of Fallot

Davlouros, Periklis; Karatza, Ageliki; Gatzoulis, Michael Α.; Shore, Darryl F.

doi:10.1016/j.ijcard.2004.08.013

Cited by 90 publications

(59 citation statements)

References 97 publications

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“…What is controversial is the timing. 12,[44][45][46][47] This issue has been hotly debated. Some believe that pulmonary valve replacement should be done only if evidence of right ventricular dysfunction has developed.…”

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confidence: 99%

When ‘blue babies’ grow up: What you need to know about tetralogy of Fallot

Fox

Devendra

Hart

et al. 2010

CCJM

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When 'blue babies' grow up: What you need to know about tetralogy of Fallot ■ AbstrActMost babies born with tetralogy of Fallot undergo corrective surgery and survive to adulthood. However, as they get older they are prone to a number of long-term problems, and they often do not receive expert-level follow-up care. This review of the adult complications of tetralogy of Fallot should help primary care practitioners identify these patients, make appropriate and timely referrals, and educate patients and their families. ■ KEY POINtsThe major long-term complication of tetralogy of Fallot repair is pulmonary valve insufficiency, which leads to right heart failure. Other problems include atrial and ventricular arrhythmias and sudden cardiac death.Surgical pulmonary valve replacement is the standard of care, but the optimal time to do this is unclear.Novel and experimental therapies include percutaneous pulmonary valve replacement and medical therapy with pulmonary arterial vasodilators.hildren born with tetralogy of Fallot and other congenital heart defects are living longer-long enough for new problems to arise, and, eventually, to present to your clinic. In primary care, the presentation of tetralogy of Fallot is still rare, but it is becoming more common.Congenital heart disease was once solely a pediatric specialty, but adults who have been treated for these conditions now outnumber children with congenital heart conditions. [1][2][3][4] More than 85% of infants with congenital heart disease are now expected to reach adulthood. 5,6 For those with tetralogy of Fallot, the most common form of cyanotic congenital heart disease, the 40-year survival rate is now at least 90%. 5But these former "blue babies" eventually have serious problems. Most develop pulmonary valve insufficiency (regurgitation), which, over time, can result in right ventricular volume overload, enlargement, and dysfunction.7-10 These problems lead to arrhythmias, the most significant cause of illness and death in these patients.11-13 Ventricular and atrial arrhythmias occur in up to 35% of patients with tetralogy of Fallot, and over a follow-up period of up to 30 years the incidence of sudden cardiac death is 6%. 14 Furthermore, because many patients have no symptoms in early adulthood, they are often lost to follow-up, potentially missing the opportunity to have complications treated before they become irreversible. Recent data suggest that most patients who present with symptoms had stopped seeing a cardiologist about 10 years before.15 rEVIEW

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confidence: 99%

When ‘blue babies’ grow up: What you need to know about tetralogy of Fallot

Fox

Devendra

Hart

et al. 2010

CCJM

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“…Eventually however, a significant number of patients develop dysfunction often requiring re-intervention [11,12]. The patients are teenagers or young adults, often after several previous surgical interventions.…”

Section: Discussionmentioning

confidence: 99%

“…Those patients usually had undergone several surgical interventions. The general indications for pulmonary valve replacement are well defined [1,2] and rely on right ventricular (RV) enlargement and functional compromise, along with pulmonary artery stenosis and/or regurgitation. Both are preferentially presented on magnetic resonance imaging [3].…”

Section: Introductionmentioning

confidence: 99%

Immediate and long-term outcomes of percutaneous transcatheter pulmonary valve implantation

et al. 2017

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“…However, in older childhood and adulthood, morbidity and mortality occurs secondary to RV dysfunction, arrhythmias, and sudden death (9). Without pulmonary valve (PV) replacement, irreversible RV dysfunction may occur (10).…”

Section: Introductionmentioning

confidence: 99%

CMR assessment of right ventricular function in patients with combined pulmonary stenosis and insufficiency after correction of tetralogy of Fallot

et al. 2013

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Background: Tetralogy of Fallot (TOF) is one of the most common types of congenital heart disease and requires prompt surgical correction. Post-correction pulmonary insufficiency (PI) often ensues in adulthood. At times, the PI is accompanied by residual pulmonary stenosis (PS). Little is known regarding right ventricular (RV) function in the setting of combined PS and PI. Purpose: To compare cardiac magnetic resonance (CMR) parameters for the assessment of RV function between patients with combined pulmonary stenosis and pulmonary insufficiency (PSPI) and isolated PI following surgical repair of TOF. Material and Methods: Retrospective review of patients with comparable corrected TOF and similar PI was performed. Seventeen patients (median age, 24 years; range, 10-52 years) had combined PSPI and 30 patients (median age, 30 years; range, 6-70 years) had isolated PI. Cine magnetic resonance (MR) images (Philips Medical Systems, Best, The Netherlands) in the short-axis plane were used to calculate end-systolic, end-diastolic, and stroke volumes (RVESV, RVEDV, RVSV) and to measure RV wall thickness. Velocity-encoded cine MR images were used to measure pulmonary regurgitation fraction (PRF) by calculating the ratio of backward flow and total forward flow, obtained from the main pulmonary flow analysis. Peak pressure gradient across the pulmonary valve was obtained from spectral Doppler echocardiography. Results: RVEF was 51 AE 8% in the PSPI patients and 39 AE 11%, in the patients with isolated PI (P ¼ 0.001). Additionally, RV wall thickness was 5.2 AE 0.8 mm in the PSPI patients compared to 2.6 AE 0.9 mm in the isolated PI patients (P ¼ 0.001). RVESVi and RVEDVi were significantly lower (P < 0.05) in patients with combined PSPI (60 AE 21 mL/m 2 , 121 AE 35 mL/m 2 , respectively) compared to the patients with isolated PI (95 AE 48 mL/m 2 , 152 AE 61 mL/m 2 , respectively). Conclusion: RV function is preserved in patients with PSPI when compared to patients with PI following surgical repair of TOF.

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Timing and type of surgery for severe pulmonary regurgitation after repair of tetralogy of Fallot

Cited by 90 publications

References 97 publications

When ‘blue babies’ grow up: What you need to know about tetralogy of Fallot

When ‘blue babies’ grow up: What you need to know about tetralogy of Fallot

Immediate and long-term outcomes of percutaneous transcatheter pulmonary valve implantation

CMR assessment of right ventricular function in patients with combined pulmonary stenosis and insufficiency after correction of tetralogy of Fallot

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