左心低形成症候群

BACKGROUND: We evaluated the surgical outcomes of the rapid two-stage Norwood procedure for hypoplastic left heart syndrome (HLHS) and its variants, focusing on low birth weight. METHOD: From October 2010 to September 2023, 67 consecutive children with HLHS or HLHS variants underwent bilateral pulmonary artery banding (bil-PAB). They were categorised into Group S (birth weight <2,500 g) and Group L (birth weight ≥2,500 g). Clinical perioperative data were analysed. RESULTS: There were no inter-stage deaths or patients requiring comfort care in either group. The in-hospital mortality rate for the Norwood procedure was 23.8% in Group S and 4.5% in Group L (p=0.031). At the time of the procedure, patients in Group S were older (p<0.001) and had a longer time from bil-PAB (p<0.001) and lower weight (p=0.013). Overall survival was significantly better in Group L than in Group S (5-year survival: Group L, 72%; Group S, 48%, p=0.037). CONCLUSIONS: The rapid two-stage Norwood procedure yielded excellent results for HLHS and its variants, particularly in patients with greater body weight who had a 4.5% in-hospital mortality rate. Despite challenging outcomes for those with birth weights <2,500 g, the strategy of allowing a waiting period and further weight gain before the Norwood procedure may still offer a viable alternative to comfort care.

OBJECTIVES: Heart failure induced by systemic ventricular dysfunction after the Norwood procedure may improve with medications. This study aimed to evaluate the incidence of heart failure, medication use, and subsequent improvement after the Norwood procedure. METHODS: Among the patients undergoing the Norwood procedure between 2001 and 2024, those who demonstrated heart failure (systemic ventricular dysfunction lasting > 30 days) were identified. Medical therapy and clinical outcomes were evaluated in relation to the recovery of systemic ventricular dysfunction. RESULTS: Among 380 patients after the Norwood procedure, heart failure was observed in 55 patients. Recovery of systemic ventricular function occurred in 21 patients with a median duration of 187 (interquartile range: 77-354) days. The cumulative incidence of recovery was 60.9% at 5 years. Recovery was more frequently observed in patients whose onset was between stage II and III palliation than in other periods (58% vs. 28%, p = .029). Survival after the onset of heart failure was better in patients who showed recovery than in those who did not (88.8% vs. 26.7% at 5 years, p < .001). At the onset of heart failure, zlog-N-terminal prohormone of brain natriuretic peptide (4.2 ± 1.3 vs. 4.5 ± 1.3, p = .55) was similar but differed at the last follow-up (2.2 ± 1.3 vs. 5.1 ± 1.5, p = .01) in patients with and without recovery. A phosphodiesterase type 5 inhibitor was identified as a factor that promoted the recovery (hazard ratio: 5.89, p < .01). CONCLUSIONS: In patients who developed heart failure after the Norwood procedure, survival was better in those with recovered function than in those with sustained dysfunction. Phosphodiesterase type 5 inhibitors were identified as a factor promoting the recovery.

BACKGROUND: Digoxin use after the Norwood procedure has been associated with improved interstage survival in hypoplastic left heart syndrome and related conditions. Whether this benefit translates into improved longer-term outcomes through staged palliation remains unknown. We aimed to determine the association of digoxin use at Norwood discharge with transplant-free survival and Fontan completion. METHODS: We conducted a retrospective cohort study using the Pediatric Heart Network (PHN) Single Ventricle Reconstruction trial public dataset, including 549 infants enrolled at 15 North American centers between 2005 and 2008. Competing risk analysis was used to evaluate Fontan completion and Cox regression to assess death or transplantation within 6 years after the Norwood procedure. Mixed-effects models compared pre-Fontan hemodynamic and echocardiographic right ventricular indices between patients treated with and without digoxin after accounting for center clustering and adjustment for sex, shunt type, heart failure medications at Norwood discharge, and census block poverty level. RESULTS: The 6-year cumulative incidence of Fontan completion was higher among patients discharged on digoxin than among those not receiving digoxin (82% vs 71%; p = 0.013). Competing-risk analysis accounting for death and transplant demonstrated a greater likelihood of Fontan completion among digoxin users (aHR 1.31; 95%CI 1.09-1.58; p = 0.005), without significant difference in the hazard of death or transplant (aHR 0.78; 95%CI 0.53-1.15; p = 0.208). No significant differences in pre-Fontan hemodynamic or echocardiographic indices were observed between groups. Initiation of digoxin post Stage II procedure was not associated with improved survival or likelihood to complete Fontan. CONCLUSION: Digoxin use at the time of Norwood discharge was associated with a 30% greater likelihood of Fontan completion by 6 years, without accompanying improvement in transplant-free survival. These findings extend prior observations of improved interstage outcomes associated with digoxin use and suggest that treatment may facilitate progression through staged palliation. CLINICAL PERSPECTIVE: What is new?: 1)Digoxin use during the interstage was associated with a greater likelihood of reaching Fontan completion within 6 years after Norwood discharge. However, this association was not accompanied by differences in right ventricular hemodynamic or echocardiographic indices at the time of Fontan evaluation.What are the clinical implications?: 2)These findings extend prior observations of improved interstage outcomes associated with digoxin use and provide important information for clinicians caring for patients with single-ventricle heart disease. Further studies are needed to clarify the mechanisms underlying this association and to identify patients most likely to benefit.

BACKGROUND: Optimal development of the pulmonary arteries (PAs) is crucial for Fontan circulation. This study investigated longitudinal changes in PA size in patients who underwent staged Fontan palliation and analyzed their impact on outcomes. METHODS: Serial PA angiograms at bidirectional cavopulmonary shunt (BCPS), extracardiac total cavopulmonary connection (TCPC), and after TCPC were reviewed. PA index (PAI), right and left PAI, and PA symmetry index were calculated, and serial changes were compared. RESULTS: Among 391 patients, right BCPS was performed in 328 (83.9%). The median age at TCPC was 2.1 years (interquartile range: 1.7-2.6 years). The most frequent diagnosis was hypoplastic left heart syndrome, in 148 patients (37.9%). PAI did not change between BCPS and TCPC (P = .564) or between pre-TCPC and post-TCPC (P = .569); however, right PAI increased between BCPS and TCPC (P = .007), while left PAI decreased (P < .001). Neither right PAI (P = .341) nor left PAI (P = .413) changed significantly between pre-TCPC and post-TCPC. The PA symmetry index decreased between BCPS and TCPC (P < .001) but remained stable thereafter (P = .912). Pre-TCPC small PAI predicted prolonged pleural effusion (P = .009; odds ratio [OR], 0.995) and ascites (P = .005; OR, 0.990). Pre-TCPC low symmetry index was a risk factor for mortality (P = .032; hazard ratio, 12.930), and its cutoff of 0.61 discriminated transplantation-free survival (P = .006). CONCLUSIONS: During the staged Fontan procedure, the left PAI decreased, whereas the PAI and right PAI remained stable or increased. A PA symmetry index <0.61 predicts mortality following TCPC. This progressive asymmetry in left-to-right PA size affects in-hospital morbidity and emphasizes the importance of achieving balanced PA growth prior to Fontan completion.

BACKGROUND: In hypoplastic left heart syndrome with aortic atresia, the non-functional left ventricle may influence right ventricular performance. We evaluated serial changes in tricuspid and mitral valve diameters after the Norwood procedure and their relationship to right ventricular function. METHODS: Of 150 patients who underwent the Norwood procedure (2001-2020), 47 died before total cavopulmonary connection. Of the 103 survivors, 76 with sufficient serial echocardiographic data were analysed (1,399 examinations). Tricuspid and mitral valve diameters were measured, and right ventricular function was assessed. Right ventricular dysfunction was defined as any degree of impairment. Mixed-effects models were used to account for repeated measures. RESULTS: Median follow-up was 4.6 years (IQR 2.4-11.8). Right ventricular dysfunction was observed in 15.9% of examinations. Patients with right ventricular dysfunction had significantly larger tricuspid valve diameters than those with normal function (24.3 mm vs 23.4 mm, p = 0.006), whereas mitral valve diameters showed no significant difference (7.6 mm vs 7.1 mm, p = 0.120). The mitral/tricuspid ratio was similar between groups (0.320 versus 0.324, p = 0.340). Mixed-effects modelling showed that the tricuspid valve diameter remained 0.618 mm larger in the dysfunction group after adjusting for growth over time (p = 0.029). Tricuspid valve diameter differences emerged at the bidirectional cavopulmonary shunt stage and accelerated through Fontan completion (p < 0.001). CONCLUSIONS: In hypoplastic left heart syndrome with aortic atresia, right ventricular dysfunction is associated with progressive tricuspid valve enlargement but not with changes in the mitral/tricuspid ratio. While the stable ratio suggests proportional annular growth, a comprehensive volumetric assessment is needed to clarify ventricular-ventricular interactions.