Twin reversed arterial perfusion (TRAP) sequence, is a rare malformation occurring in monozygotic multiple gestations. One well-developed normal (pump) twin and the other twin with absent cardiac structure (acardiac), who is hemodynamically dependent on the normal (pump) twin are characteristic of this syndrome. The acardiac twin develops multiple anomalies that make survival difficult. The prognosis of the pump twin is variable with mortality rate ranging from 50% to 70%. Complications that affect the prognosis of the pump twin include complications of congestive cardiac failure due to increased cardiac demand, prematurity secondary to preterm delivery, and polyhydramnios. Because of these complications prompt detection, follow-up, and treatment of this condition is very important. We report two cases of TRAP sequence that emphasizes the importance of gray-scale and color Doppler imaging in diagnosis, detection of poor prognostic features, follow-up, and management of TRAP sequence.
Aims
Excessive prolongation of PR interval impairs coupling of atrio‐ventricular (AV) contraction, which reduces left ventricular pre‐load and stroke volume, and worsens symptoms. His bundle pacing allows AV delay shortening while maintaining normal ventricular activation. HOPE‐HF evaluated whether AV optimized His pacing is preferable to no‐pacing, in a double‐blind cross‐over fashion, in patients with heart failure, left ventricular ejection fraction (LVEF) ≤40%, PR interval ≥200 ms and either QRS ≤140 ms or right bundle branch block.
Methods and results
Patients had atrial and His bundle leads implanted (and an implantable cardioverter‐defibrillator lead if clinically indicated) and were randomized to 6 months of pacing and 6 months of no‐pacing utilizing a cross‐over design. The primary outcome was peak oxygen uptake during symptom‐limited exercise. Quality of life, LVEF and patients' holistic symptomatic preference between arms were secondary outcomes. Overall, 167 patients were randomized: 90% men, 69 ± 10 years, QRS duration 124 ± 26 ms, PR interval 249 ± 59 ms, LVEF 33 ± 9%. Neither peak oxygen uptake (+0.25 ml/kg/min, 95% confidence interval [CI] −0.23 to +0.73, p = 0.3) nor LVEF (+0.5%, 95% CI −0.7 to 1.6, p = 0.4) changed with pacing but Minnesota Living with Heart Failure quality of life improved significantly (−3.7, 95% CI −7.1 to −0.3, p = 0.03). Seventy‐six percent of patients preferred His bundle pacing‐on and 24% pacing‐off (p < 0.0001).
Conclusion
His bundle pacing did not increase peak oxygen uptake but, under double‐blind conditions, significantly improved quality of life and was symptomatically preferred by the clear majority of patients. Ventricular pacing delivered via the His bundle did not adversely impact ventricular function during the 6 months.
This case series highlights the rare but potentially life threatening complication of ventricular perforation caused by pacemaker leads and discusses appropriate investigations and management strategies.
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