Relation between right ventricular enlargement, QRS duration, and right ventricular function in patients with tetralogy of Fallot and pulmonary regurgitation after surgical repair

Rahman, Mohamed Y. Abd El; Abdul-Khaliq, H.; Vogel, Michael; Alexi-Meskishvili, Vladimir; Gutberlet, Matthias; Lange, Peter

doi:10.1136/heart.84.4.416

Cited by 140 publications

(80 citation statements)

References 20 publications

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“…Furthermore, a QRS duration greater than 180 ms is a significant marker of risk of ventricular arrhythmias and sudden death. 22,[34][35][36][37] Another feature strongly associated with ventricular arrhythmias and sudden death is the rate of change in the QRS duration. A relatively rapid increase (> 3.5 ms/year) is associated with a significantly higher risk.…”

Section: ■ Findings On Assessmentmentioning

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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Section: ■ Findings On Assessmentmentioning

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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“…These alterations may decrease RV ejection fraction. 8 The motion of the interventricular septum often becomes paradoxic in these circumstances, indicating an abnormal RV activation sequence. 9 Thus, RV dyskinesis appears to be a reasonable therapeutic target.…”

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confidence: 99%

Electrical Resynchronization

et al. 2003

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Background-Many patients with congenital heart disease develop right ventricular (RV) failure due to anatomy and prior therapy. RV problems may include right bundle-branch block (RBBB), volume loading, and chamber enlargement. Because the failing RV may have regional dyskinesis, we hypothesized that resynchronization therapy might augment its performance. Methods and Results-We studied 7 patients with RV dysfunction and RBBB, using a predefined pacing protocol. QRS duration, cardiac index (CI), and RV dP/dt were measured in 4 different pacing states. Atrioventricular pacing improved CI and RV dP/dt max and decreased QRS duration as compared with atrial pacing or sinus rhythm. Conclusions-Atrioventricular pacing in patients with RBBB and RV dysfunction augments RV and systemic performance. RV resynchronization is a promising novel therapy for patients with RV failure. (Circulation. 2003;107:2287-2289.)

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“…Continuous exposure to pulmonary regurgitation leads to progressive RV dilation and dysfunction, therefore initiating adverse clinical outcomes (arrhythmias, heart failure, sudden cardiac death) [7,40,41]. PR is consistent with transannular patch use in TOF patients, a technique predominantly applied in the 20th century to relieve RVOT obstruction [42].…”

Section: Residual Pulmonary Regurgitation and Right Ventricular Dilationmentioning

confidence: 85%

“…QRS with a duration of ≥180 ms is often present along with an enlarged heart (cardiothoracic ratio > 0.5), r = 0.44, p < 0.001 [10]. Another study suggests moderate correlation between QRS duration and end-diastolic volume (r = 0.6 (p < 0.01)) [41]. Association between prolonged QRS and asynchronous contraction of RVOT in patients with rTOF has been reported [58].…”

Section: Qrs Prolongationmentioning

confidence: 98%

Late arrhythmia in patients with repaired tetralogy of Fallot: who is at risk?

Grigaliūnas

Gumbienė

Valevičienė

et al. 2015

Seminars in Cardiovascular Medicine

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SummaryTetralogy of Fallot (TOF) is the most common cause of cyanotic congenital heart defect. Over the last century, the life expectancy of TOF patients has significantly improved. This, however, has brought new challenges both to patients and their health-care providers, the main of them being late arrhythmia. Ironically, late arrhythmia is predominantly generated due to the fibrotic scars caused by the life-saving surgical repair. Once the first two mainly arrhythmia-free decades after the repair pass, the risk of developing late arrhythmia and, therefore, SCD becomes substantial. Consequently, young adults with repaired Tetralogy of Fallot (rTOF) require careful outpatient monitoring.There have been many attempts to predict the risk of life-threatening arrhythmia in rTOF patients. This has led to the defining of various risk factors, ranging from the widely used QRS prolongation to novel predictors, derived from cardiac magnetic resonance (CMR) based anatomical findings (left ventricular dyssynchrony indexes, right ventricular output tract akinetic length, right ventricular mass-to-volume ratio). The latter predictors have recently established CMR as a tool of high significance in evaluation of rTOF patients.Although the role of Holter monitoring findings in rTOF patient-assessment remains unclear, it may be useful in those who are 25 years and older. Implantable cardioverter-defibrillator (ICD) implantation is the first-line treatment for secondary prevention of sudden cardiac death (SCD). rTOF patients suffer from the highest rate of inappropriate and the lowest rate of appropriate ICD shocks, when compared to other congenital heart diseases. As a consequence, ICD implantation for primary SCD prevention should be carefully weighed. Catheter-based ablation therapy leads to high rate of initial success when abolishing monomorphic ventricular tachycardia and intra-atrial reentrant tachycardia, however recurrence rates remain high. Seminars in Cardiovascular Medicine

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Relation between right ventricular enlargement, QRS duration, and right ventricular function in patients with tetralogy of Fallot and pulmonary regurgitation after surgical repair

Cited by 140 publications

References 20 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

Electrical Resynchronization

Late arrhythmia in patients with repaired tetralogy of Fallot: who is at risk?

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