Implantation techniques and outcomes after cardiac resynchronization therapy for congenitally corrected transposition of the great arteries.

BACKGROUND: Patients with congenitally corrected transposition of the great arteries (CCTGA) are at risk of congestive heart failure (CHF). There are limited data on cardiac resynchronization therapy (CRT) techniques and long-term outcomes in this population. OBJECTIVE: The purpose of this study was to determine implantation techniques and efficacy of CRT for CCTGA. METHODS: A 15-year retrospective review of patients with CCTGA undergoing CRT was performed. RESULTS: Twenty patients were identified (mean age 40.1 ± 15.3 years; baseline New York Heart Association class 2.0 [interquartile range 1.5-3.5]). Indication was pacing-induced ventricular dysfunction in 12 (60%), atrioventricular block with anticipation for >40% ventricular pacing in 5 (25%), and intact atrioventricular conduction with CHF and QRS prolongation in 3 (15%). A transvenous approach was successful in 18 of 19 patients (95%) in whom it was attempted, with cannulation of a posteroseptal ostium in 14 (78%), vein of Marshall in 2 (11%), and superior ectopic ostium in 2 (11%). Of patients with baseline CHF, 8 (67%) were acute responders, with loss of response in 2 patients (median 1.1 and 1.5 years, respectively). Of 4 patients referred for heart transplantation, 3 (75%) could be de-listed following CRT. Only lead location in the right ventricular outflow tract predicted poor CRT response (P = .026). Post-procedure, the QRS duration increased by 4.3 ms/y (P < .001) despite stable pacing characteristics. CRT revision was required in 4 patients for infection (n = 2) or phrenic nerve capture (n = 2) and was associated with loss of CRT response in 1 patient. CONCLUSION: A transvenous approach to CRT involving distinct coronary venous patterns is feasible for most patients with CCTGA anatomy. Long-term outcome is favorable, but is characterized by return of right ventricular dysfunction in some patients.

Incidence and outcome of infective endocarditis following percutaneous versus surgical pulmonary valve replacement.

OBJECTIVES: To provide a comparison of the outcome of infective endocarditis (IE) in patients undergoing transcatheter pulmonary valve replacement (TPVR) versus surgical pulmonary valve replacement (SPVR). BACKGROUND: Although TPVR is thought to be associated with a higher risk of IE than SPVR, there is paucity of data to support this. METHODS: Patients who underwent TPVR or SPVR at UCLA between October 2010 and September 2016 were included and retrospectively analyzed. RESULTS: Three hundred forty-two patients underwent PVR at UCLA including 134 SPVR and 208 TPVR. Patients undergoing TPVR were more likely to have had a history of endocarditis than those undergoing SPVR (5.3% vs. 0.7%, P = 0.03) and a right ventricle to pulmonary artery (RV to PA) conduit (37% vs. 17%, P = 0.0001). Two SPVR and seven TPVR patients developed IE with a 4-year freedom from endocarditis of 94.0% in the SPVR versus 84% in the TPVR group (P = 0.13). In patients who underwent TPVR and developed endocarditis, the mean gradient across the RVOT prior to intervention was higher (28.1 ± 4.5 vs. 17.4 ± 0.6 mmHg, P = 0.02) and were more likely to have a conduit (71% vs. 36%, P = 0.049). CONCLUSIONS: In this study, patients undergoing TPVR were not at a higher risk of IE than patients undergoing SPVR. TPVR patients were more likely to have had a prior history of IE and RV-PA conduit. The patients at highest risk were those with stenotic RV to PA conduits who were treated with TPVR.

Mortality Risk Stratification in Fontan Patients Who Underwent Heart Transplantation.

The number of patients who require orthotopic heart transplantation (OHT) for failing Fontan physiology continues to grow; however, the methods and tools to evaluate risk of OHT are limited. This study aimed to identify a set of preoperative variables and characteristics that were associated with a greater risk of postoperative mortality in patients who received OHT for failing Fontan physiology. Thirty-six Fontan patients were identified as having undergone OHT at University of California-Los Angeles Medical Center from 1991 to 2014. Data were collected retrospectively and analyzed. The primary end point was designated as postoperative mortality. After an average follow-up time of 3.5 years, 17 (44%) patients suffered postoperative mortality. Patient characteristics including (1) age <18 years at the time of OHT, (2) Fontan-OHT interval of <10 years, (3) systemic ventricular ejection fraction <20%, (4) moderate-to-severe atrioventricular valve insufficiency, (5) an elevated Model of End-stage Liver Disease, eXcluding INR score, or (6) need for advanced mechanical support before surgery were associated with an increased incidence of postoperative mortality. Using these risk factors, we present a theoretical framework to stratify risk of postoperative death in failing Fontan patients after OHT. In conclusion, a method such as this may aid in the transplantation evaluation and listing process of patients with failing Fontan physiology.

Transcatheter melody valve placement in large diameter bioprostheses and conduits: What is the optimal "Landing zone"?

OBJECTIVES: This study sought to elucidate the optimal bioprosthetic valve (BPV) size prior to Melody valve implantation. BACKGROUND: BPVs provide an ideal "landing zone" for future Melody valve insertion. To guide surgical choice of BPV size, it is important to understand which BPV size can serve consistently as substrates for Melody valve placements. METHODS: A database of all patients who underwent Melody implantation at UCLA or Kaiser Permanente Los Angeles from 2010 to 2014 was analyzed retrospectively. Patients with an existing BPV were stratified into those with a valve diameter of ≥27 mm or <27 mm. RESULTS: One hundred and sixty patients underwent catheterization with the intention to implant a Melody valve. Melody valve implantation was performed in the pulmonary position in 52 patients with prior BPVs. The immediate procedural success rate was 100%. Immediately post-Melody, the right ventricular to pulmonary artery gradient was significantly higher in the <27 mm group compared to the ≥27 mm group (14.3±3 vs. 8.6±6.8, P=0.006). There was a significantly shorter time from prior valve replacement to Melody implantation in the <27 mm group. There was one patient in whom transcatheter pulmonary valve implantation was aborted due to inadequate landing zone in the <27 mm group, and no patients in the ≥27 mm group (P=NS). CONCLUSIONS: The results of this study indicate that 27 and 29 mm BPV provide a superior landing zone for Melody valve implantation with excellent immediate and intermediate term hemodynamic results when compared to smaller BPVs less than 27 mm.

Pheochromocytoma and paraganglioma in cyanotic congenital heart disease.

CONTEXT: Aberrant cellular oxygen sensing is a leading theory for development of pheochromocytoma (PHEO) and paraganglioma (PGL). OBJECTIVE: The objective of the study was to test the hypothesis that chronic hypoxia in patients with cyanotic congenital heart disease (CCHD) increases the risk for PHEO-PGL. DESIGN/SETTING/PARTICIPANTS: We investigated the association between CCHD and PHEO-PGL with two complementary studies: study 1) an international consortium was established to identify congenital heart disease (CHD) patients with a PHEO-PGL diagnosis confirmed by pathology or biochemistry and imaging; study 2) the 2000-2009 Nationwide Inpatient Survey, a nationally representative discharge database, was used to determine population-based cross-sectional PHEO-PGL frequency in hospitalized CCHD patients compared with noncyanotic CHD and those without CHD using multivariable logistic regression adjusted for age, sex, and genetic PHEO-PGL syndromes. RESULTS: In study 1, we identified 20 PHEO-PGL cases, of which 18 had CCHD. Most presented with cardiovascular or psychiatric symptoms. Median cyanosis duration for the CCHD PHEO-PGL cases was 20 years (range 1-57 y). Cases were young at diagnosis (median 31.5 y, range 15-57 y) and 7 of 18 had multiple tumors (two bilateral PHEO; six multifocal or recurrent PGL), whereas 11 had single tumors (seven PHEO; four PGL). PGLs were abdominal (13 of 17) or head/neck (4 of 17). Cases displayed a noradrenergic biochemical phenotype similar to reported hypoxia-related PHEO-PGL genetic syndromes but without clinical signs of such syndromes. In study 2, hospitalized CCHD patients had an increased likelihood of PHEO-PGL (adjusted odds ratio 6.0, 95% confidence interval 2.6-13.7, P < .0001) compared with those without CHD; patients with noncyanotic CHD had no increased risk (odds ratio 0.9, P = .48). CONCLUSIONS: There is a strong link between CCHD and PHEO-PGL. Whether these rare diseases coassociate due to hypoxic stress, common genetic or developmental factors, or some combination requires further investigation.