Clinical Evaluation of Exercise Capacity in Adults with Systemic Right Ventricle.

The right ventricle provides systemic circulation in individuals with congenitally corrected transposition of the great arteries (CCTGA) and in those with complete transposition who have had an atrial switch repair (DTGA). The aim of this study was to evaluate how the systemic right ventricle adapts to increased workload and oxygen demand during exercise. From November 2005 through December 2015, 3,358 adult patients with congenital heart disease were treated at our institution; we identified 48 (26 females, 22 males; median age, 25.4 ± 8.1 yr) who met the study criteria; 37 had DTGA and atrial switch repair, and 11 had CCTGA. We studied their echocardiographic and cardiopulmonary exercise test results. A control group consisted of 29 healthy sex- and age-matched volunteers. On exercise testing, oxygen uptake at anaerobic threshold, peak oxygen uptake, peak heart rate, and percentage of maximal heart rate were significantly lower in the group with systemic right ventricle than in the control group (all P <0.001); in contrast, the peak ventilatory equivalent for carbon dioxide was higher in the study group (P=0.013). Impaired systemic right ventricular function reduced peak oxygen uptake. The peak heart rate was lower in the CCTGA group than in the DTGA group. Our results indicate that reduced exercise capacity is related to impaired systemic right ventricular function, severe tricuspid valve regurgitation, and chronotropic incompetence. There was no correlation between cardiopulmonary exercise test results and time after surgery. Chronotropic efficiency is lower in individuals with CCTGA than in those with DTGA.

Long-term observation of adults after successful repair of aortic coarctation.

INTRODUCTION: Despite successful repair of aortic coarctation, cardiovascular complications occur. AIM: To analyse type and frequency of late complications and their impact on exercise capacity in adults after aortic coarctation repair. MATERIAL AND METHODS: Fifty-eight adults after aortic coarctation repair, 36 male, median age 27.46 ±10.57, were compared to 30 healthy volunteers. Physical examination, transthoracic echocardiography, carotid intima-media thickness measurement, cardiopulmonary exercise test and 24-hour ambulatory blood pressure monitoring were performed. RESULTS: The main complications were: arterial hypertension 48.3%, myocardial hypertrophy in echocardiography 29.34%, recoarctation 25.86%, aortic dilation 13.79% and coronary artery disease 6.89%. Exercise tolerance was reduced in the cardiopulmonary exercise test. The VO2/kg peak was lower, 29.01 ±8.79 vs. 49.16 ±7.38 ml/kg/min, p < 0.001, VE/VCO2 peak higher 28.18 ±4.69 vs. 26.78 ±3.13, p = 0.017. The peak heart rate was reduced, 157.28 ±22.22 vs. 177.93 ±23.08 bpm, p < 0.001, peak systolic blood pressure was higher, 174.79 ±17.62 vs. 153.33 ±4.79 mm Hg, p < 0.001. Systolic blood pressure in 24-hour ambulatory monitoring correlated with left ventricle mass index, r = 0.29, p = 0.025, wall thickness, r = 0.31, p = 0.039. Age at operation was related to left ventricle wall thickness, r = 0.27, p = 0.041, and carotid intima-media thickness, r = 0.26, p = 0.046. There was no association of any cardio-pulmonary parameters with time from surgery, type of operation or echocardiography results. CONCLUSIONS: Adults after aortic coarctation repair suffer from arterial hypertension, recurrent aortic stenosis, aortic aneurysms, and coronary artery disease. Reduced exercise capacity in cardio-pulmonary exercise test is related to hypertensive reaction and chronotropic incompetence.

[Cardiovascular risk factors in patients after coarctation of aorta repair]. / Czynniki ryzyka chorób sercowo-naczyniowych u chorych po leczeniu interwencyjnym koarktacji aorty.

The coarctation of the aorta (CoA) is a congenital condition of the thoracic aorta. AIM: The aim of the study was assessment of atherosclerosis risk factors in adult patients after surgical treatment of aortic coarctation. MATERIALS AND METHODS: 58 patients (36 male, 22 female) at median age of 27.46 ±10.57 were compared with 30 healthy, age and sex matched volunteers. The arterial blood pressure, lipid profile, fasting glucose, inflammation markers and the lifestyle factors were analyzed. RESULTS: CoA patients have higher systolic blood pressure 136.55±16.27 vs 123.47±10.34 mmHg, p<0.001, fasting glucose 4.95±0.5 vs 4.65±0.46 mmol/l p=0.002, hsCRP 1.03±0.12 vs 0.89±0.14 mg/l p=0.025 and fibrinogen 2.55±0.34 vs 1.98±0.28 g/l p<0.001. Hyperlipidemia is more common 44.8% vs 23.3% p=0.048, treated with statin. When comparing hypertensive patients (N=28) with normotensive ones (N=30), the patients with arterial hypertension are older 33.5±12.23 vs 25.73±7.12 p=0.004, have higher body weight 78.03±14.58 vs 68.7±14.29 p=0.017, in this group more common are: hypoplastic aortic arch 28.6% vs 6.7% p=0.027, recoarctation 39.3% vs 13.3% p=0.024, cardiovascular disease 14.3% vs 0% p=0.032 and family history 21.4% vs 3.3% p=0.034. CONCLUSIONS: The coarctation of aorta is related to higher cardiovascular risk due to arterial hypertension, hyperlipidemia, higher glucose and inflammation markers levels when comparing with healthy population.

Long-Term Effects of Percutaneous Fenestration Following the Fontan Procedure in Adult Patients with Congenital Univentricular Heart.

BACKGROUND The Fontan procedure, performed for univentricular heart, may also include the technique of percutaneous fenestration to create a small atrial septal defect (ASD) and a right-to-left shunt. The aim of this study was to evaluate the long-term effects of fenestration in adult patients who had a Fontan procedure for univentricular heart. MATERIAL AND METHODS Fontan surgery was performed in 39 patients, including 19 (49%) patients with fenestration (Group I), and 20 (51%) patients without the fenestration procedure (Group II). Laboratory tests in both groups included echocardiography, plethysmography, cardiopulmonary exercise testing, and 24-hour Holter monitoring. RESULTS Compared with patients in Group I, patients in Group II had a significantly increased level of N-terminal pro-brain natriuretic peptide (NT-proBNP) (p=0.04), alkaline phosphatase (ALP) (p=0.01) and a significant increase in frequency of atrial fibrillation (p=0.04). Patients in Group I had a significantly increased systemic ventricular ejection fraction (SVEF) (p=0.05) and increased heart rate (HR) (p=0.006), heart rate reserve (HRR) (p=0.02), ventilatory equivalent (VE) (p=0.01), and VO2 peak (p=0.05) on cardiopulmonary exercise testing (CPET). Renal, hematologic, and ventilatory parameters, and incidence of thromboembolism showed no significant differences between the groups. CONCLUSIONS Long-term follow-up of patients who underwent Fontan procedures with percutaneous fenestration had improved single ventricular function, lower NT-proBNP levels, improved exercise capacity, and reduced ALP levels. These findings indicate that percutaneous fenestration closure should be considered for adult patients who have undergone Fontan procedure for univentricular heart.

[Multiorgan malfunction after Fontan operation in adult patients]. / Powiklania narzadowe u doroslych chorych po operacji Fontana.

Although 85% of patients with univentricular heart after Fontan procedure survive twenty years after operation, the procedure alone seems to be an inadequate treatment as a permanent clinical solution. Patients with a "Fontan physiology" additionally have to face a various extra-cardiac complications, including thyroid, liver and kidney dysfunction, which are not only potentially life-threatening, but also can potentiate the circulatory insufficiency. AIM: The aim of the study was to assess a multiorgan dysfunction in adult patients after Fontan operation in long term follow-up and compare to healthy controls. MATERIALS AND METHODS: 54 patients after Fontan procedure (age=25.1±7 years, time after operation =19.8±6.3 years, age at the time of the procedure =5.3±4.3 years) and 30 controls (26.2±5.8 years) were included to the study. Clinical and laboratory examinations were performed including: hematologic, hepatic, renal and thyroid function tests. The following laboratory tests were performed: red blood cell counts (RBC), hematocrit (Ht), hemoglobin (Hb), platelets count, red blood cell distribution width (RDW), iron level; Btype natriuretic peptide (NT-proBNP), proteinogram blood test, alanine aminotransferase (ALT), aspartate aminotransferase (AST), gammaglutamyl transpeptidase (GGTP), alkaline phosphatase (ALP), total bilirubin, alpha fetoprotein (AFP) level. Furthermore creatinine level; cystatin C, urine albumin to creatynine ratio (ACR) and urinalysis were assessed. To assess a thyroid function free triiodothyronine (fT3), free thyroxine (fT4) and thyroid stimulating hormone (TSH) were measured. Furthermore an abdomen ultrasonography was performed. RESULTS: In terms of the hematological disorders Fontan patients had a statistically significant higher level of RBC (5.6±0,8 vs 4.8±0.4 109/µl; p<0,001), Hb (16.5±2.8 vs 14.2±1.2 g/dl; p<0,001), HCT (48.7±8.1 vs 42.1±3 %; p<0,001), RDW (14±2.6 vs 12.8±0.5 %; p=0.001), as compared to control group, while PLT level was statistically lower in Fontan group (156.2±61.4 vs 224.2±48 103/µl; p<0,0001). Hepatic parameters in Fontan patients were statistically significant higher, as depicted by the level of: ALT (28.5±10.5 vs 21.5±6 U/l, p<0,001), GGTP (85.6±48.8 vs 19.3±9 U/ l, p<0,001), total bilirubin (26.6±24.8 vs 8.9±4.7 µmol/l; p<0,001), ALP (82.4±31.5 vs 51.2±16 U/l; p<0,001) and INR (1.21±0.3 vs 0.98±0.2; p<0,001). Other parameters such as: AFP, total protein and albumin level did not statistically significantly differ. Additionally five patients (9%) according to clinical symptoms and serum albumin level were diagnosed protein-losing enteropathy (PLE). There was no difference in serum creatinine level between Fontan patients and control group (79.6±23.6 vs 75.9±18.9 µmol/l; p=0.8) as well as in uric acid level (342.7±102 vs 303±105 µmol/l; p=0.2). Cystatin C level was significantly higher in Fontan group in comparison to controls (1.1±0.6 vs 0.8±0.1 ng/ml; p=0,05). Furthermore four Fontan patients (7%) had abnormal microalbumin/creatynine ratio. Urine testing preformed in 30 Fontan patients pointed to 7 cases (23%) where hematuria was observed, while proteinuria was detected in 4 cases (13%) and urobilinogen in 2 (7%). Dysfunction of the thyroid gland was found in 30% of patients after Fontan operation (7% in the control group, p=0,034). Among disorders of the thyroid gland: 13% of patients were diagnosed with hyperthyroidism, 54% diagnosed with subclinical hypothyroidism, and the remaining 33% with hypothyroidism. CONCLUSIONS: Our study show that adult patients after Fontan procedure in long-term follow are exposed to multiorgan complications including hematological, liver, kidney and thyroid dysfunction.