Determinants and prognostic value of albuminuria in adult patients with congenital heart disease.

BACKGROUND: The determinants and prognostic value of albuminuria remain unclear in patients with adult congenital heart disease (ACHD), especially in those with Fontan circulation (FC). METHODS: We retrospectively reviewed 512 consecutive ACHD patients and investigated the determinants of urinary albumin-to-creatinine ratio (ACR) and albuminuria (MAU) and their association with all-cause mortality. Demographic data and laboratory and hemodynamic parameters were collected. Regression analysis and Cox proportional hazard models were used to identify the relationship between log ACR and variables, and clinical factors and all-cause mortality, respectively. RESULTS: Body mass index, aortic systolic blood pressure (ASP), arterial oxygen saturation (SaO2), glycated hemoglobin (HbA1c), B-type natriuretic peptide, and diuretic use were independently associated with log ACR. ASP, SaO2, and HbA1c were independently associated with MAU (P < .05-0.001). The prevalence of MAU was highest in unrepaired patients with low SaO2 (50%; P < .0001). Log ACR and MAU were associated with exercise capacity and all-cause mortality (P < .0001 for both) independent of renal function. Patients with ACHD, MAU, and renal dysfunction (n = 23) had the highest risk of all-cause mortality, while those without MAU or renal dysfunction had the lowest risk (P < .0001). These prognostic values remained significant in separate analyses of Fontan and biventricular circulation (P < .0001). CONCLUSIONS: ASP, SaO2, and HbA1c levels were independently associated with MAU in ACHD patients. MAU and log ACR were associated with all-cause mortality in patients with Fontan and biventricular circulation, independent of renal dysfunction.

Prevalence and clinical correlates and characteristics of "Super Fontan".

BACKGROUND: Super-Fontan (SF) is an excellent phenotype of patients with Fontan circulation and normal exercise capacity. This study aimed to clarify the prevalence and clinical correlates and characteristics of SF. METHODS: We reviewed 404 Fontan patients who had undergone cardiopulmonary exercise testing, and the results were compared with clinical profiles. RESULTS: Seventy-seven (19%) patients had SF, and the postoperative prevalence at 5, 10, 15, 20, and ≥ 25 years was 16 (35%), 30 (39%), 18 (19%), 13 (14%), and 0 (0%), respectively. Compared with non-SF, SF patients were younger (P < .001) and were mostly men (P < .05). SF was characterized by a current high arterial blood pressure and oxygen saturation (SaO2), low systemic ventricle (SV) end-diastolic pressure, favorable body composition, superior pulmonary function, preserved hepatorenal and hemostatic functions, and better glucose tolerance (P < .05-.001). Pre-Fontan better SV function, low pulmonary artery resistance, and high SaO2 were associated with current SF (P < .05-.01). Furthermore, positive trajectory of exercise capacity and high daily activity during childhood were associated with current adult SF (P < .05). During the follow-up, 25 patients died, and 74 patients were unexpectedly hospitalized. There was no death in the SF group, and the rate of hospitalization was 67% lower than that of the non-SF group (P < .01-.001). CONCLUSIONS: The prevalence of SF gradually decreased over time. SF was characterized by preserved multi-end-organ function and an excellent prognosis. Pre-Fontan hemodynamics and post-Fontan childhood daily activity were associated with being adult SF.

Thirty-year outcome in children with hypertrophic cardiomyopathy based on the type.

BACKGROUND: We reviewed the long-term outcome of children with hypertrophic cardiomyopathy (HCM) based on the type. METHODS: We reviewed the medical records of 100 patients (male 54 female 46) with HCM at our hospital between 1977 and 2015. The survival and cardiac event-free survival rates were calculated by the Kaplan-Meier method. RESULTS: The age at the time of the diagnosis ranged from 0 to 15 years with a median of 8 years. The number of patients with Noonan syndrome and hypertrophic obstructive cardiomyopathy (HOCM), idiopathic HCM (i-HCM), and secondary HCM (s-HCM) was 13, 13, 65, and 9 respectively. A dilated phase of HCM occurred in 24 patients. Nineteen (79 %) of the 24 patients died of heart failure, and two underwent a heart transplantation. Eight (33 %) of the 24 patients had s-HCM. The median age when a dilated phase occurred was 15 years old, and the median interval from the initial diagnosis to the dilated phase was 8 years. The median time from the diagnosis of a dilated phase to death was 1.6 years. Sudden death and implantable cardioverter defibrillator implantations occurred in 6 and 11 patients at around 15 years old, respectively. The 20-year survival rates were as follows: Noonan syndrome 84 %; HOCM 82 %; i-HCM 71 %; and s-HCM 17 %. Overall, the survival rates at 10, 20, and 30 years were 83 % (95 % confidence interval 73-89), 69 % (58-78), and 63 % (50-74), respectively. The overall cardiac event-free survival rates at 10, 20, and 30 years were 57 % (47-67), 39 % (31-50), and 32 % (21-44), respectively. CONCLUSION: The long-term outcome in children with HCM was poor, and the outcome of s-HCM was very poor. The occurrence of a dilated phase worsened the outcome in HCM patients. Sudden death and d-HCM often occurred at around 15 years old.

Pulmonary Arteriovenous Fistulae After Fontan Operation: Incidence, Clinical Characteristics, and Impact on All-Cause Mortality.

Background: The Fontan operation is a surgical procedure used in children with univentricular hearts. Pulmonary arteriovenous fistulae (PAVF) is a major complication after a Fontan operation. However, the incidence and related clinical pathophysiology of PAVF remain unclear. Purpose: This study aimed to clarify the incidence of PAVF, its clinical characteristics, and its influence on all-cause mortality. Methods and Results: We serially assessed the presence of PAVF using pulmonary artery angiography and/or contrast echocardiography during catheterization in 391 consecutive patients who underwent the Fontan procedure and compared the results with the Fontan pathophysiology and all-cause mortality. PAVF developed in 36 patients (9.2%), including 30 diffuse- and six discrete-PAVF types. The PAVF-free rates at 1, 5, 10, 15, 20, and ≥25 years after Fontan operation were 97, 96, 93, 88, 87, and 83%, respectively. The mean arterial blood oxygen saturation (SaO2) in patients with diffuse PAVF at each corresponding postoperative stage were 90, 91, 91, 91, 89, and 88%, respectively, indicating lower SaO2 levels than those in patients without PAVF (all p < 0.01). However, there was no difference in the SaO2 levels between patients with discrete PAVF and those without PAVF. During a median follow-up period of 2.9 years after the last catheterization, 31 patients, including 12 patients with PAVF, died. Patients with PAVF, especially those with diffuse PAVF, had a higher mortality rate (p = 0.01) than those without PAVF (hazard ratio: 3.6, 95% confidence interval: 1.6-7.8, p = 0.0026). Conclusion: Patients who underwent Fontan surgery had an increased incidence of PAVF as they aged. Discrete PAVF did not influence SaO2 or mortality, whereas the presence of diffuse PAVF caused hypoxia and was associated with all-cause mortality.

Clinicopathologic Study of Placentas From Women With a Fontan Circulation.

BACKGROUND: Pregnant women with a Fontan circulation have a high risk of obstetric complications, such as preterm delivery and small for gestational age (SGA), which may be affected by low blood flow to the placenta and hypoxia. This study investigated placental pathology in a Fontan circulation.Methods and Results:Eighteen pregnancies in 11 women with a Fontan circulation were reviewed. Pregnancy outcomes showed 9 miscarriages and 9 live births, with 4 preterm deliveries. Five neonates were SGA (<5th percentile). Eight placentas from live births in 7 women were available for the study. Five placentas had low weight placenta for gestational age, and 7 grossly showed a chronic subchorionic hematoma. Histological examination revealed all placentas had some form of histological hypoxic lesions: maternal vascular malperfusion in 7, fetal vascular malperfusion in 1, and other hypoxia-related lesions in 8. Quantitative analyses, including immunohistochemistry (CD31, CD68, and hypoxia inducible factor-1α antibodies) and Masson's trichrome staining, were also performed and compared with 5 control placentas. Capillary density and the area of fibrosis were significantly greater in placentas from women with a Fontan circulation than in control placentas. CONCLUSIONS: Placentas in a Fontan circulation were characterized by a high frequency of low placental weight, chronic subchorionic hematoma, and constant histological hypoxic changes, which could reflect altered maternal cardiac conditions and lead to poor pregnancy outcomes.

Impact of bilateral bidirectional Glenn anastomosis on staged Fontan strategy and Fontan circulation.

OBJECTIVES: The aim of this study was to identify the impact of bilateral bidirectional Glenn (BBDG) anastomosis on staged Fontan strategy and late Fontan circulation. METHODS: Of 267 patients who underwent bidirectional Glenn prior to Fontan completion between 1989 and 2013, 62 patients (23%) who underwent BBDG were enrolled in this study. Age at operation was 0.84 years (25th-75th percentile: 0.58-1.39). Thirty-three patients had heterotaxy syndrome (53%). The mean follow-up period was 12.7 ± 8.1 (max. 30.6) years. RESULTS: The overall survival rate at 15 years was 73%. Although 49 patients (79.0%) went on to Fontan completion, 12 patients (19.4%) died without achieving it. Thrombus formation and poor development in a central pulmonary artery were not observed, but obstruction of the superior vena cava (SVC) occurred in 8 patients (13%), mainly those with right atrial isomerism (P = 0.037). SVC obstruction was not, however, a risk factor for mortality (P = 0.097) or Fontan completion (P = 0.41). The shape of BBDG anastomosis, symmetricity of pulmonary blood flow, impingement of caval blood flow returning from the superior and inferior vena cavae or coexisting interrupted inferior vena cava with azygos or hemi-azygos continuation did not affect late Fontan outcomes, such as overall survival, freedom from protein-losing enteropathy or pulmonary arterio-venous malformation rates. CONCLUSIONS: SVC obstruction after BBDG frequently occurred, mainly in patients with right atrial isomerism; however, its direct impact on prognosis or achieving Fontan completion was not identified. Once Fontan circulation was established, the arrangement of the Fontan pathway did not affect late Fontan outcomes.

Long-Term Outcomes After Fenestration Closure in High-Risk Fontan Candidates.

The study aimed to assess the long-term outcomes after fenestration closure in patients at risk for Fontan failure. Of 119 patients who underwent Fontan operation between 1995 and 2004, fenestration was not created in 89 patients (NF group) and created in 30 patients with hypoplastic left heart syndrome, heterotaxy syndrome, high pulmonary arterial pressure, high systemic ventricular end-diastolic pressure, low ventricular ejection fraction, or atrioventricular valve regurgitation. All fenestrations were closed spontaneously or by catheter/surgical interventions, excepting two patients, and therefore, they were excluded. In fenestration group, patients with pre-Fontan mean pulmonary arterial pressure ≥ 15 mmHg or systemic atrioventricular valve regurgitation ≥ moderate were classified as high-risk Fontan candidates (F-HR group, n = 16), and the remaining patients were as standard-risk (F-SR group, n = 12). Protein-losing enteropathy-free survival rates did not differ among the three groups (p = 0.72). Serial follow-up catheter examinations after Fontan operation were completed in 69 patients in NF group and 11 patients in both F-SR and F-HR groups. Cardiac index and pulmonary vascular resistance significantly and similarly decreased over time in all groups, though the F-HR group showed lowest arterial oxygen saturation, lowest cardiac index, and highest pulmonary vascular resistance. The F-HR group also showed much veno-venous collaterals (p = 0.049), low peak oxygen consumption (p = 0.019), and low anaerobic threshold (p = 0.023) as compared to those in the F-SR group. In F-HR group, cyanosis remained after fenestration closure due to transformation from fenestration to veno-venous collaterals, which resulted in elevation of pulmonary vascular resistance, low cardiac index, and deterioration of exercise tolerance.

Morphological changes and number of candidates for transcatheter pulmonary valve implantation in conduits involving heterograft and artificial material.

Heterograft and artificial materials have been used for extracardiac conduit implantation to create right ventricular (RV) to pulmonary artery (PA) continuity for biventricular repair in Japan because of the limited availability of homograft valves. However, few studies have examined morphological changes and number of candidates for transcatheter pulmonary valve implantation (TPVI) in which the conduit includes more than one type of material. Overall, 88 patients who underwent biventricular repair with an external conduit were included in this evaluation. Based on catheterization data and surgical records, we estimated morphological change in the RV outflow tract for each material and the number of candidates for Melody valve implantation based on premarket approval application criteria established by the U.S. Food and Drug Administration. There were 63 candidates for TPVI (72%, 63/88). Median anteroposterior and lateral diameter of the RV outflow tract was 20.4 mm (range 9.0-41.5) and 17.8 mm (range 9.5-34.9), respectively. Bovine pericardium tended to dilate by 11.2%. Polytetrafluoroethylene (ePTFE), homograft, and Dacron polyethylene terephthalate (PET) tended to become stenotic by 11.1%, 28.0%, and 13.4%, respectively. While ePTFE (27/33, 82%) and Dacron PET (2/2, 100%) were highly suitable for TPVI, bovine pericardium (32/48, 67%) was less suitable. In Japan, many patients with hemodynamic indications for TPVI following extracardiac conduit implantation to create RV to PA continuity may also meet the morphological indications.

The reduced left ventricular stroke volume does not fully recover after pulmonary valve replacement in patients with repaired tetralogy of Fallot.

OBJECTIVES: The present study was conducted to investigate the decrease in left ventricular stroke volume index (LVSVI) that is caused by pulmonary regurgitation-induced right heart dysfunction and its clinical implications before and after pulmonary valve replacement (PVR). METHODS: Between January 2010 and December 2019, 30 adults who underwent surgical PVR for chronic pulmonary regurgitation with right ventricular dilation late after tetralogy of Fallot (TOF) repair were included. All patients were evaluated using cardiac magnetic resonance before PVR. The median interval from TOF repair to PVR was 29 [25th, 75th percentile: 25, 37] years. The median pulmonary regurgitation fraction and right ventricular end-diastolic volume index were 56 [48, 66] % and 203 [187, 239] ml/m2. Twenty-three patients (76.7%) were re-evaluated 1 year after PVR. RESULTS: Before PVR, the median LVSVI was 40 [35, 46] ml/beat/m2. A lower LVSVI was associated with a longer interval from TOF repair to PVR (r = -0.40, P = 0.029) and a lower right ventricular ejection fraction (r = 0.52, P = 0.004). A lower LVSVI was not associated with a higher right ventricular end-diastolic volume index. LVSVI remained unchanged after PVR. The patients were subdivided into Normal-stroke volume index (SVI) and Subnormal-SVI groups using the preoperative LVSVI cut-off value of 35 mL/beat/m2. Compared with the Normal-SVI group, the Subnormal-SVI group had a higher incidence of ablation therapy before PVR (4.7 vs 2.3 patient-years, P = 0.044). After PVR, LVSVI in the Subnormal-SVI group was still lower (40 [34, 42] vs 44 [42, 47] ml/beat/m2, P = 0.038) despite the right ventricular end-diastolic volume index normalization. There was no difference in the clinical event incidence between the 2 groups during the follow-up period. Brain natriuretic peptide level in the Subnormal-SVI group was higher within 3 years after PVR (P = 0.046). CONCLUSIONS: Reduced left ventricular stroke volume did not fully recover after PVR. PVR for patients with repaired TOF should be performed before the left ventricular stroke volume begins to decrease.

Incidence, Predictors, and Mortality in Patients With Liver Cancer After Fontan Operation.

Background Liver cancer (LC) is a serious late complication after the Fontan operation. However, the incidence, predictors, and prognosis remain unknown. The purpose of our study was to determine these clinical characteristics. Methods and Results We assessed liver function in 339 consecutive patients who had undergone the Fontan procedure from 2005 to 2019. LC was histologically diagnosed in 10 patients after a median period of 2.9 years (range: 0.3-13.8; median age: 29.9 years [range: 14.4-41.5 years]; overall median post-Fontan procedure follow-up: 25.6 years [range: 13-32.1 years]), and the annual incidence was 0.89%. Over the entire post-Fontan follow-up period, the annual incidences of new-onset LC in the second, third, and fourth decades were 0.14%, 0.43%, and 8.83%, respectively. The patients with LC had longer follow-up periods, higher levels of AFP (α-fetoprotein), and higher values of liver fibrosis indices (P<0.01-0.0001). Moreover, all indices were predictive of new-onset LC (P<0.01-0.0001). The LC treatments were surgical resection (n=3), transarterial chemoembolization (n=3), radiofrequency ablation (n=2), and hospice care (n=2). During a median follow-up of 9.4 months, 4 patients died; the survival rate at 1 year was 60%, and it was better among asymptomatic patients (P<0.01). Conclusions The LC incidence rapidly increased ≥30 years after the Fontan procedure, and liver fibrosis indices and AFP were predictive of new-onset LC. These LC-predictive markers should be monitored closely and mandatorily for early LC detection and better prognosis.

Systemic Artery Vasoconstrictor Therapy in Fontan Patients with High Cardiac Output-Heart Failure: A Single-Center Experience.

Failed Fontan Patients with high cardiac output (CO) heart failure (HF) might have vasodilatory syndrome and markedly high mortality rates. The aim of this study was to review the clinical effects of vasoconstrictor therapy (VCT) for failed Fontan hemodynamics. We retrospectively reviewed 10 consecutive patients with Fontan failure (median age, 33 years) and high CO-HF who had received VCT. The hemodynamics were characterized by high central venous pressure (CVP: median, 16 mm Hg), low systolic blood pressure (median, 83 mm Hg), low systemic vascular resistance (median, 8.8 U·m2), high cardiac index (median, 4.6 L/min/m2), and low arterial oxygen saturation (median, 89%). VCT included intravenous noradrenaline infusion for five unstable patients, oral midodrine administration for nine stable patients, and both for four patients. After VCT introduction with a median interval of 1.7 months, the median systolic blood pressure (102 mm Hg, p = 0.004), arterial oxygen saturation (90%, p = 0.03), and systemic vascular resistance (12.1 U·m2, p = 0.13) increased without significant changes in CVP or cardiac index. After a median follow-up of 21 months, the number of readmissions per year decreased from 4 (1-11) to 1 (0-9) (p = 0.25), and there were no VCT-related complications; however, five patients (50%) developed hepatic encephalopathy, and six patients (60%) eventually died. VCT was safely introduced and could prevent the rapidly deteriorating Fontan hemodynamics. VCT could be an effective therapeutic strategy for failed Fontan patients with high CO-HF.

Electrocardiographic features of arrhythmogenic right ventricular cardiomyopathy in school-aged children.

It can be difficult to distinguish children with early-stage arrhythmogenic right ventricular cardiomyopathy (ARVC) from those with benign premature ventricular contraction (PVC). We retrospectively evaluated six school-aged children with ARVC and compared with those of 20 with benign PVC. The median age at initial presentation was 11.4 and 10.2 years in ARVC and benign PVC, respectively. None of the ARVC patients fulfilled the diagnostic criteria of ARVC at initial presentation. At ARVC diagnosis, the treadmill exercise test and Holter monitoring showed provoked PVC during exercise and pleomorphic PVC in all ARVC cases, respectively. During the observation period, terminal activation duration (TAD) was prolonged in all ARVC patients. In addition, ΔTAD (5.5 [3-10] ms) were significantly longer than those with benign PVC (p < 0.001). A new notched S-wave in V1 appeared in four (67%) ARVC patients, who had myocardial abnormalities in the right ventricle, and in zero benign PVC. Our electrocardiographic findings, such as provoked PVC during exercise, pleomorphic PVC, prolonged TAD, and a new notched S-wave in V1 could contribute to the early detection of ARVC in school-aged children.

Exercise-induced hypoxia predicts hypobaric hypoxia during flight in patients after Fontan operation.

BACKGROUND: Hypobaric hypoxia (HH) during flight might be more detrimental to pulmonary circulation in Fontan patients compared healthy individuals. This study was designed to clarify whether exercise-induced hypoxia could predict HH during flight in Fontan patients. METHODS AND RESULTS: Percutaneous oxygen saturation (SpO2) was analyzed during flight in 11 Fontan patients and eight volunteers. SpO2 was measured before taking off (S1), at the initial (S2), the end of stabilization (S3), and after landing (S4). The SpO2-dynamics were compared with SpO2-dynamics during cardiopulmonary exercise testing (CPX), pulmonary function, and hemodynamics in the Fontan patients. At all measurements, SpO2 was lower in the Fontan patients than the volunteers during flight. The total SpO2 decline from S1 to S3 was greater in the Fontan patients than the volunteers. While SpO2 change from S2 to S3 was negative in the Fontan patients, it was stable in the volunteers. In the Fontan patients, the median value of exercise-induced SpO2 decline (Ex-dSpO2), SpO2 at rest, and SpO2 at peak was -6%, 93%, and 88%, respectively. In addition to exercise capacity and pulmonary function, the Ex-dSpO2 was correlated strongly with SpO2 at all phases during flight (r = 0.75-0.98, p < 0.01 for all). Flight-associated adverse events occurred in two patients with SpO2 < 80% at S3. CONCLUSIONS: Both the Fontan patients and the volunteers demonstrated similar SpO2-dynamics during flight with a greater HH in the Fontan patients. CPX with SpO2 monitoring is useful in predicting SpO2-dynamics and adverse events during flight in these patients.

Impact of delayed ventricular wall area ratio on pathophysiology of mechanical dyssynchrony: implication from single-ventricle physiology and 0D modeling.

Electrical disparity can induce inefficient cardiac performance, representing an uncoordinated wall motion at an earlier activated ventricular wall: an early shortening followed by a systolic rebound stretch. Although regional contractility and distensibility modulate this pathological motion, the effect of a morphological factor has not been emphasized. Our strain analysis in 62 patients with single ventricle revealed that those with an activation delay in 60-70% of ventricular wall area suffered from cardiac dysfunction and mechanical discoordination along with prolonged QRS duration. A computational simulation with a two-compartment ventricular model also suggested that the ventricle with an activation delay in 70% of the total volume was most vulnerable to a large activation delay, accompanied by an uncoordinated motion at an earlier activated wall. Taken together, the ratio of the delayed ventricular wall has a significant impact on the pathophysiology due to an activation delay, potentially highlighting an indicator of cardiac dysfunction.

Etiology of atrial fibrillation in patients with complex congenital heart disease - for a better treatment strategy.

BACKGROUND: The demographics of patients with congenital heart disease (CHD) and atrial fibrillation (AF) differ significantly from the general population. The etiology and treatment strategy for AF in CHD patients have been investigated but are to date inconclusive. METHODS: To determine the etiology of AF in CHD and to seek a better treatment strategy, we retrospectively evaluated the atrial overload in 42 complex CHD cases with normal atrial arrangements and AF (age 25; range, 9-66 years) and the impact of a reduction in the atrial overload on the atrial rhythm. RESULTS: Cardiac defect diagnoses varied, with 17% of the patients having a persistent left superior vena cava (PLSVC). In regard to the volume overload, the frequencies of an overload in the right atrium (RA), left atrium (LA), or both, were 50 %, 23%, and 10%, respectively (p = 0.015). Other sustained supraventricular tachycardias were observed in 29 patients (69%) before and after the onset of AF. Among these 29 patients, 26 had intra-atrial reentrant tachycardia. Fifteen patients (36%), 10 of whom had chronic AF, died during the follow-up including 3 with arrhythmias and 10 because of heart failure. Fourteen (33%) patients had no AF at the last follow-up due to medical interventions, 8 of which underwent solely an RA-sided catheter ablation and/or surgical RA overload reduction. CONCLUSIONS: AF in complex CHD with a normal atrial arrangement correlates with a higher RA-sided overload than an LA-sided and exhibits a high incidence of PLSVCs, high comorbidity of intra-atrial reentrant tachycardias, and high mortality rate. In a substantial number of patients, RA-sided interventions were effective in controlling AF. To effectively manage AF in complex CHD it is essential to understand each individual's hemodynamics and consider hemodynamic interventions.

Effect of Stiffened and Dilated Ascending Aorta on Aerobic Exercise Capacity in Repaired Patients With Complex Congenital Heart Disease.

Several studies have reported aortic dilation and increased stiffness of the ascending aorta in patients after repair of congenital heart disease (CHD), which may be a predominant cardiovascular risk. However, the clinical significance has not been described in detail. In this retrospective study, 175 repaired patients with complex CHD achieving biventricular circulation and age-matched 39 control subjects were reviewed (median age: 14.9 and 15.7 years, respectively). We measured the diameters of the ascending aorta and descending aorta from catheterization angiograms to yield Z-scores and stiffness indexes (ß) using diameter fluctuations corresponding to pulsatile pressures. Clinical profile, peak oxygen uptake during the cardiopulmonary exercise test, and incidence of unscheduled hospitalization during follow-up was also reviewed. Compared with controls, patients with complex CHD, except for those with aortic coarctation, exhibited significant dilation and increased stiffness of the aortic root and ascending aorta, but not of the descending aorta. In this CHD population (n = 147, including 112 conotruncal anomalies), exercise capacities correlated independently with the diameter Z-score and stiffness index of the ascending aorta along with the history of repetitive thoracotomies, reduced forced vital capacity, and right ventricular hypertension. During a follow-up period (median 15.6 years), either dilation (Z-score >3.5) or increased stiffness (ß >6.0) of the ascending aorta stratified morbidity, but no synergistic impact was detected. In conclusion, in repaired patients with complex CHD, a stiffened and dilated ascending aorta was frequently found, exerting significant adverse impacts on diminished exercise capacity and morbidity.