Comparison of long-term outcomes of atrial switch with Rastelli and physiological repair using left ventricle-to-pulmonary artery conduit for levo-transposition of the great arteries.

OBJECTIVES: The objectives of this study was to compare the long-term outcomes of anatomic repair using atrial switch with the Rastelli procedure versus physiological repair with left ventricle-to-pulmonary artery conduit for patients with levo-transposition of the great arteries, ventricular septal defect, and left ventricular outflow tract obstruction. METHODS: Of patients with levo-transposition of the great arteries who underwent biventricular repair between 1978 and 2001, 31 hospital survivors after anatomic repair of atrial switch and the Rastelli (anatomic group) and 14 hospital survivors after physiological repair with left ventricle-to-pulmonary artery conduit (physiological group) were enrolled. Survival rates, reoperation rates, and most recent conditions were compared. RESULTS: The overall survival rate at 20 years was 79.7% (95% CI, 66.4%-95.6%) in the anatomic group and 85.1% (95% CI, 68.0%-100%) in the physiological group (P = .87). The reoperation rate at 10 years was 19.8% (95% CI, 5.6%-34.0%) in the anatomic group and 52.0% (95% CI, 25.0%-79.1%) in the physiological group (P = .067). Only patients in the physiological group underwent systemic tricuspid valve replacement. The anatomic group showed a better cardiac index at catheterization (2.79 ± 0.75 L/min/m2 vs 2.30 ± 0.54 L/min/m2; P = .035), lower serum brain natriuretic peptide (73 ± 86 pg/mL vs 163 ± 171 pg/mL; P = .024), and better maximal oxygen uptake in the treadmill test (64.1 ± 16.5% vs 52.7 ± 17.8% of predicted normal; P = .036), although the period until most recent catheterization, blood inspection, and treadmill testing were earlier in the anatomic group. CONCLUSIONS: Preservation of the left ventricle as the systemic ventricle using anatomic repair contributes to better cardiopulmonary condition compared with physiological repair.

Anaesthetic management of a large paraganglioma resection in a woman with isolated L-looped transposition of the great arteries: a case report.

BACKGROUND: Reports of anaesthetic management of paraganglioma resection in patients with isolated L-transposition of the great arteries (L-TGA) are rare. We focus on the preoperative evaluation, intraoperative management, and postoperative care of a frail patient with "physiologically corrected" L-TGA for paraganglioma resection. CASE PRESENTATION: We performed general anaesthesia for a 46-year-old patient with "physiologically corrected" L-TGA undergoing open large retroperitoneal paraganglioma resection. Although the preoperative medical therapy had attained its goals, the patient went through three periods of severe episodic hypertension and tachycardia as tumour manipulation released catecholamines. Goal-directed fluid therapy based on pulse pressure variation (PPV) and point-of-care transesophageal echocardiography (TEE) imaging enabled anaesthesiologists to make rapid judgments and to regulate blood pressure in a timely manner, thereby reducing the risk of heart failure caused by massive rapid fluid bolus therapy. The patient was transferred to the intensive care unit because of intraoperative hemodynamic changes and significant blood loss. Despite transient myocardial injury (elevated troponin I), no lethal arrhythmia or complications occurred perioperatively, and the patient recovered well and was discharged 1 week later. CONCLUSIONS: Goal-directed fluid therapy combined with the adoption of TEE could effectively guide fluid administration, which is helpful for anaesthesia management during operation. We recommend the routine use of TEE in such cases.

Late recovery of sinus rhythm following perioperative complete atrioventricular block in a child with congenitally corrected transposition.

We report a patient with l-transposition of the great arteries who developed perioperative complete atrioventricular block at 5 years of age in conjunction with a modified double-switch operation, but had unexpected late recovery of normal sinus rhythm months later. This case highlights that even for patients with l-transposition, which is particularly vulnerable to developing both spontaneous and perioperative complete atrioventricular block, late recovery of atrioventricular node conduction may still be possible after surgery.

From tricuspid to double orifice Morphology: Percutaneous tricuspid regurgitation repair with the MitraClip device in congenitally corrected-transposition of great arteries.

Edge to edge transcatheter mitral valve repair with MitraClip (Abbott Vascular, Menlo Park, CA) is increasing for high-risk surgical patients with significant mitral regurgitation. Patients with congenitally corrected transposition of the great arteries (CCTGA) presenting with tricuspid valve regurgitation of a systemic right ventricle may represent particularly challenging candidates for MitraClip given their anatomy. We report the case of a 67-year-old gentleman with CCTGA and severe tricuspid regurgitation who was referred for MitraClip implantation after heart team consensus. Successful implantation of one clip was performed, achieving a significant reduction of the regurgitation. Similarly, favorable findings were confirmed at 6 months, 1 and 2 years follow-up and the patient had no recurrent heart failure admissions after 2-year follow-up. We describe the technical considerations and the importance of 3D-transoesophageal echocardiography for performing the MitraClip of a trileaflet systemic atrioventricular valve. © 2016 Wiley Periodicals, Inc.

Modern Incidence of Complete Heart Block in Patients with L-looped Ventricles: Does Univentricular Status Matter?

OBJECTIVE: Individuals with L-transposition of the great arteries and two normally sized ventricles are at risk for complete heart block. Little is known about the incidence of complete heart block in those with a single ventricle L-transposition of the great arteries. In this study, we compare the incidence of complete heart block in a modern cohort of patients with L-looped single ventricle anatomy to patients with L-transposition of the great arteries and two ventricles. METHODS: We conducted a retrospective cohort study of patients with L-transposition of the great arteries who were seen at Yale-New Haven Hospital between 2001 and 2013. Patients were classified as having isolated L-transposition of the great arteries (group I), L-transposition of the great arteries and major cardiac defects with two-ventricle anatomy (group II), or L-transposition of the great arteries and single ventricle anatomy (group III). We recorded the age of onset and the circumstances of CHB in each group. We calculated the incidence rate of complete heart block and compared this between the groups. RESULTS: We identified 64 patients with L-transposition of the great arteries, median age of 21 years (range 6 months-52 years): 21 in group I, 15 in group II, and 28 in group III. In total, 15 subjects developed complete heart block, incidence of 21.9% and rate of 1.3% per person years. Although group III patients were significantly less likely to develop complete heart block than dual ventricle patients (7.1% vs. 33% and 40%, P = .01), this difference is not significant when only spontaneous complete heart block was analyzed (P = .16). CONCLUSION: All patients with L-transposition of the great arteries have similar risk of spontaneous complete heart block and should be routinely screened for this complication.

A typical case of L-transposition of the great arteries initially presented as complete atrioventricular block in middle-aged man.

L-transposition of the great arteries (L-TGA) is a rare congenital anomaly and could cause complete atrioventricular (AV) block at relatively younger age. We present a case of 43-year-old male who complained of dizziness due to complete AV block. We confirmed L-TGA using transthoracic echocardiography and cardiac computed tomography. Permanent pacemaker was inserted without complications. No invasive treatment including corrective surgery was performed because patient's cardiac function was almost normal and the symptom was completely resolved after pacemaker insertion.

A Typical Case of L-Transposition of the Great Arteries Initially Presented as Complete Atrioventricular Block in Middle-Aged Man

L-transposition of the great arteries (L-TGA) is a rare congenital anomaly and could cause complete atrioventricular (AV) block at relatively younger age. We present a case of 43-year-old male who complained of dizziness due to complete AV block. We confirmed L-TGA using transthoracic echocardiography and cardiac computed tomography. Permanent pacemaker was inserted without complications. No invasive treatment including corrective surgery was performed because patient's cardiac function was almost normal and the symptom was completely resolved after pacemaker insertion.