Olga Trojnarska

The BNP concentrations and exercise capacity assessment with cardiopulmonary stress test in cyanotic adult patients with congenital heart diseases

Cyanosis is observed in patients with complex congenital heart disease (CHD) and pulmonary hypertension, heart failure represents an important clinical problem in such patients. The aim of this study was to evaluate the exercise capacity in patients with cyanotic CHDs using cardiopulmonary exercise test, measuring serum BNP levels as well as to seek correlation between BNP levels and cardiopulmonary exercise test parameters and identify the effects of blood oxygen desaturation and pulmonary hypertension on these indices. The study group consisted of 53 patients (21 males) at the mean age of 39.4 ± 14.3 years, of whom 19 were operated on at the mean age of 9.6 ± 8.6 years. Mean blood oxygen saturation (SO(2)) in patients was 81.2 ± 6.2%. Twenty four patients presented with Eisenmenger syndrome, 16--univentricular hearts, 4--transposition of the great arteries, 6--Fallots tetralogy, and 3--Ebstein anomaly. The control group comprised 32 healthy individuals (16 males) at the mean age of 40.7 ± 9.9 years. Cardiopulmonary stress test showed significantly lower exercise capacity in patients with cyanosis than in controls: maximal oxygen uptake (VO(2max)) 15.5 ± 4.9 vs. 31.6 ± 7.1 ml/kg/min (p=0.00001), maximum heart rate at peak exercise (HR max): 139.5 ± 22.5 bpm vs. 176.6 ± 12.1 (p=0.0001), VE/VCO(2) slope: 46.4 ± 10.1 vs. 27.3 ± 2.9 (p=0.00001), forced vital capacity FVC: 3.1 ± 1.1 l vs. 4.4 ± 0.8 l (p=0.00001). Subjects with the evidence of pulmonary hypertension (PH+) had lower exercise capacity than those without (PH-): VO(2max): 17.2 ± 4.2 vs. 12.8 ± 4.8 ml/kg/min (p=0.002), VE/VCO(2): 43.7 ± 11.1 vs. 50.9 ± 6.4 (p=0.01), FVC: 3.46 ± 1.05 l vs. 2.37 ± 0.91 l (p=0.0002). Plasma BNP levels in the study group were higher than in controls: 122.4 ± 106.7 vs. 21.1 ± 20.2 pg/ml p=0.00001 and did not differ between PH+ and PH- groups (115.7 ± 99.0 vs. 127.9 ± 114.1 pg/ml p=0.78). Negative correlations between BNP levels and VO(2max) (r=-0.389, p=0.006), FVC (r=-0.395 p=0.005), FEV1 (r=-0.386 p=0.006), SO(2) (r=-0.445 p=0.00001), and positive correlation between BNP level and VE/VCO(2) (r=0.369 p=0.009) were found.

Evaluation of exercise capacity with cardiopulmonary exercise testing and BNP levels in adult patients with single or systemic right ventricles.

Introduction The aim of the study was to evaluate exercise capacity using cardiopulmonary exercise test (CpET) and serum B-type natriuretic peptide (BNP) levels in patients with single or systemic right ventricles. Material and methods The study group included 40 patients (16 males) – 17 with transposition of the great arteries after Senning operation, 13 with corrected transposition of the great arteries and 10 with single ventricle after Fontan operation, aged 19–55 years (mean 28.8 ±9.5 years). The control group included 22 healthy individuals (10 males) aged 23–49 years (mean 30.6 ±6.1 years). Results The majority of patients reported good exercise tolerance – accordingly 27 were classified in NYHA class I (67.5%), 12 (30%) in class II, and only 1 (0.5%) in class III. Cardiopulmonary exercise test revealed significantly lower exercise capacity in study patients than in control subjects. In the study vs. control group VO2max was 21.7 ±5.9 vs. 34.2 ±7.4 ml/kg/min (p = 0.00001), maximum heart rate at peak exercise (HRmax) 152.5 ±32.3 vs. 187.2 ±15.6 bpm (p = 0.00001), VE/VCO2 slope 34.8 ±7.1 vs. 25.7 ±3.2 (p = 0.00001), forced vital capacity (FVC) 3.7 ±0.9l vs. 4.6 ±0.3 (p = 0.03), forced expiratory volume in 1 s (FEV1) 3.0 ±0.7 vs. 3.7 ±0.9l (p = 0.0002) respectively. Serum BNP concentrations were higher in study patients than in control subjects; 71.8 ±74.4 vs. 10.7 ±8.1 (pg/ml) respectively (p = 0.00001). No significant correlations between BNP levels and CpET parameters were found. Conclusions Patients with a morphological right ventricle serving the systemic circulation and those with common ventricle physiology after Fontan operation show markedly reduced exercise capacity. They are also characterized by higher serum BNP concentrations, which do not however correlate with CpET parameters.

Evaluation of Exercise Capacity with Cardiopulmonary Exercise Testing and Type B Natriuretic Peptide Concentrations in Adult Patients with Patent Atrial Septal Defect

Adults with patent atrial septal defect (ASD) usually find their exercise capacity satisfactory, and therefore hesitate to accept proposed surgical treatment of the heart disease. The aim of our study was to evaluate both the exercise capacity, using the cardio-pulmonary stress test, and brain natriuretic peptide (BNP) levels in asymptomatic adults with ASD. Thirty-six patients with patent secundum type ASD (aged mean 44.7 ± 8.2 years) were studied. The control group consisted of 25 healthy subjects at the mean age of 45.6 ± 6.1 years. Echocardiography and CPST were performed and BNP levels measured in all subjects. Oxygen uptake (VO2 max) was lower in ASD patients than in controls (22.1 ± 5.6 vs. 30.0 ± 6.8 ml/kg/min, p = 0.00001); the VE/VO2 slope was elevated in ASD patients compared with healthy subjects (31.3 ± 6.6 vs. 26.9 ± 3.3, p = 0.001), and exceeded 34 in 5 patients. VO2 max showed a negative correlation with the pulmonary to systemic flow ratio Qp:Qs (r = –0.46, p = 0.004), and a positive correlation was found between the VE/VO2 slope and Qp:Qs (r = 0.32, p = 0.05). BNP levels were higher in the ASD group than in the controls (60.6 ± 49.9 vs. 32.6 ± 24.5 pg/ml, p = 0.02). BNP correlated positively with RV diameter and Qp:Qs (r = 0.38 and 0.39 respectively, p = 0.03) and negatively with maximum VO2 (r = –0.5, p = 0.004) and VO2% (r = –0.32, p = 0.07). Conclusions: Although most adult patients with ASD perceive their exercise capacity as satisfactory, objective assessment reveals that in fact it is significantly decreased. BNP levels are increased comparing to healthy individuals. Decreased exercise capacity and increased BNP levels seem to result from right ventricular volume overload.

Arterial Stiffness in Adult Patients with Cyanotic Congenital Heart Disease

BACKGROUND Increased arterial stiffness is an important predictor of cardiovascular (CVS) morbidity and mortality. Adults suffering from cyanotic congenital heart disease (CCHD) represent a population of patients at particular risk of CVS events. Erythrocytosis is a cardinal feature of CCHD but little is known about any possible interaction between hematological derangements and arterial stiffness. METHODS Fourteen CCHD patients (6 men and 8 women, mean age 32 +/- 3 years) and 13 age-matched healthy subjects were recruited. RESULTS Mean arterial stiffness, as estimated by stiffness index of digital volume pulse (SI(DVP)) values was similar in the 2 groups (7.9 +/- 0.7 m/s vs. 7.2 +/- 0.4 m/s, P = .38). In the control subjects arterial stiffness showed a strong correlation with age and mean arterial pressure (r = 0.64, P = .02 and r = 0.58, P = .036, respectively). In patients with CCHD, arterial stiffness correlated with mean arterial pressure but not with age (r = 0.76, P = .002 and r =-0.13, P = .65, respectively). However, SI(DVP) correlated with hematocrit in CCHD patients (r = 0.69, P = .006), but not in healthy controls. The mean arterial pressure in patients with CCHD but not in controls showed a highly significant, positive correlation with the hematocrit level (r = 0.68, P = .008). CONCLUSIONS Arterial stiffness in CCHD patients is affected by arterial pressure and hematocrit concentration but did not differ significantly from that in age-matched healthy controls. It is unlikely that increased arterial stiffness may contribute to cardio- and cerebrovascular complication in adults with CCHD.

Asymmetric dimethylarginine and vascular indices of atherosclerosis in patients after coarctation of aorta repair

OBJECTIVE Cardiovascular events are the main cause of premature death after successful repair of aortic coarctation (CoA). The aim of this study was to assess the selected biochemical markers of atherosclerosis in normotensive CoA repaired patients and to establish its relation to ultrasound indexes of vascular pathology. METHODS 62 patients after CoA repair (37 males, age: 34.1 ± 1.4 yrs) and 20 control individuals (10 males, age: 34.8 ± 2.2 yrs) were enrolled in the study. The serum markers: asymmetric dimethylarginine (ADMA), nitrites/nitrates (NOx), high-sensitivity C-reactive protein (hsCRP), and following vascular parameters: flow-mediated dilatation (FMD), intima-media thickness (IMT) and pulse wave velocity (PWV) were analyzed. RESULTS 33 CoA repaired patients were normotensive, and compared to controls, they presented higher serum ADMA concentrations (0.59 ± 0.04 umol/l vs. 0.46 ± 0.03 umol/l, p=0.035). An analysis of the vascular parameters revealed decreased FMD (4.75 ± 0.5%), NMD (11.86 ± 0.8%) and increased PWV (6.90 ± 0.2 m/s) values in the normotensive patients as compared with the control group (FMD: 8.6 ± 0.9%, p<0.001, NMD: 20.94 ± 1.7%, p<0.001; PWV: 5.49 ± 0.2, p=0.023). There were no differences in the serum levels of NOx, hsCRP as well as IMT values between normotensive patients and the control group. A multivariate regression analysis revealed that serum ADMA level was a factor independently associated with the FMD value (r=-0.334; p=0.031) in normotensive CoA repaired group. CONCLUSIONS Early biochemical and vascular indices of atherosclerosis such as increased serum ADMA concentration as well as impaired vasodilatation and increased arterial stiffness are observed in patients after CoA repair. Serum ADMA is a strong predictor of endothelial dysfunction in patients with CoA repair.

Microvolt T wave alternans in adults with congenital heart diseases characterized by right ventricle pathology or single ventricle physiology: a case control study.

BackgroundAmong adults with congenital heart diseases (CHD) evaluation of sudden cardiac death (SCD) risk remains a great challenge. Although microvolt T-wave alternans has been incorporated into SCD risk stratification algorithm, its role in adults with CHD still requires investigation. We sought to determine the incidence of MTWA in this specific group and its coincidence with ventricular arrhythmia (VA) and other clinical findings presumably associated with SCD.MethodsA case–control study was performed in which 102 patients with CHD characterized by right ventricle pathology or single ventricle physiology (TGA, UVH, Ebstein’s anomaly, ccTGA, Eisenmenger syndrome, DORV, CAT, unoperated ToF) were compared to 45 age- and sex-matched controls. All subjects underwent spectral MTWA test, ambulatory ecg monitoring, cardiopulmonary test, BNP assessment. After excluding technically inadequate traces, the remaining MTWA results were classified as positive(+), negative(−) and indeterminate(ind). Due to similar prognostic significance MTWA(+) and (ind) were combined into a common group labeled ‘abnormal’.ResultsAbnormal MTWA was present more often in the study group, compared to controls (39.2% vs 2.3%, p = 0.00001). Sustained ventricular tachycardia (sVT) was observed more often among subjects with abnormal MTWA compared to MTWA(−): 19.4% vs 3.6%, p = 0.026. The patients with abnormal MTWA had a lower blood saturation (p = 0.047), more often were males (p = 0.031), had higher NYHA class (p = 0.04), worse cardiopulmonary parameters: %PeakVO2 (p = 0.034), %HRmax (p = 0.003). Factors proven to increase probability of abnormal MTWA on multivariate linear regression analysis were: sVT (OR = 20.7, p = 0.037) and male gender (OR = 15.9, p = 0.001); on univariate analysis: male gender (OR = 2.7, p = 0.021), presence of VA (OR = 2.6, p = 0.049), NYHA > I (OR = 2.06, p = 0.033), %HRmax (OR = 0.94, p = 0.005), %PeakVO2 (OR = 0.97, p = 0.042), VE/VCO2slope (OR = 1.05, p = 0.037).ConclusionsAbnormal MTWA occurs significantly more often in adults with the chosen forms of CHD than among healthy subjects. The probability of abnormal MTWA increases in patients with malignant VA, in males and among subjects with heart failure and cyanosis. MTWA might be of potential role in risk stratification for SCD in adults with CHD.

Balloon aortic valvuloplasty--ups and downs--are we facing a procedure comeback?

BACKGROUND Recently, there has been renewed interest in balloon aortic valvuloplasty (BAV). AIM To analyse the indications and short-term outcome of BAV since transcatheter aortic valve implantation (TAVI) was launched in our institution. METHODS Between September 2010 and September 2014, 25 consecutive patients (19 female, 6 male) underwent BAV. The mean age was 72 ± 11.4 years, mean EuroScore II was 10.4 ± 11.7%, mean logistic EuroScore 23.5 ± 23.6%, mean Society of Thoracic Surgeons mortality risk score was 21.8 ± 13.6%. The indications for BAV were: advanced haemodynamically unstable heart failure (HF) including cardiogenic shock or pulmonary oedema (n = 7), co-morbidities requiring urgent non-cardiac surgery (n = 8), palliative treatment (n = 6), and an intention to bridge to TAVI or aortic valve replacement in patients with severe HF (n = 4). RESULTS In-hospital mortality was 20% (n = 5) and occurred in patients who underwent BAV in the setting of haemodynamically unstable HF. Other major complications included pacemaker implantation (n = 2), major vascular complications (n = 4), and cardiac tamponade (n = 1). There were no patients who required conversion to cardiac surgery. The mean peak aortic transvalvular gradient decreased from 96.9 ± 29.5 to 60.3 ± 15.5 mm Hg (p = 0.0001) after BAV. We did not observe significant aortic regurgitation. CONCLUSIONS Treatment of advanced and haemodynamically unstable aortic stenosis, bridge to non-cardiac surgery and palliative therapy are the main reasons for BAV in recent years. BAV as a bridge to TAVI or aortic valve replacement may be an option for some patients. Short-term results are good with relatively low mortality and morbidity related to the procedure. Mortality in haemodynamically unstable patients presenting with cardiogenic shock or pulmonary oedema treated with BAV is very high.

Assessment of coagulation profile by thromboelastometry in adult patients with cyanotic congenital heart disease

BACKGROUND Patients with cyanotic congenital heart disease (CCHD) have an increased risk of bleeding and thrombotic complications. Prolonged conventional coagulation screening parameters, such as activated partial thromboplastin time or prothrombin time, are reported in less than 20% of CCHD patients. METHODS The aim of this study was to determine the haemostatic abnormalities in 32 adult patients with CCHD by rotation thromboelastometry (ROTEM) with assessment of coagulation dynamic properties, as a guide for perioperative prophylaxis or haemostatic therapy. The control group consisted of 35 healthy subjects. RESULTS Our results suggest that CCHD patients, in comparison to healthy controls, had a tendency to hypocoagulate with delayed activation of haemostasis and clot formation, initiated by both intrinsic and extrinsic activators. The growth of the clot was slower and the clot firmness was decreased, which may additionally contribute to bleeding diathesis. Moreover, the clot lysis readings suggest higher clot stability in the CCHD group. All velocity parameters were markedly lower in the CCHD patients, indicating a decreased rate of clot formation. Although coagulation tests and platelet count were normal, the usefulness of rotation thromboelastometry in monitoring or guiding therapy in CCHD patients is demonstrated. CONCLUSION In conclusion, our results provide new insights into the data on hypocoagulation with impaired clot lysis in adult CCHD patients as determined by ROTEM. Our findings may assist in determining the optimal management of patients with CCHD undergoing surgery.

ALCAPA syndrome in a 56-year-old woman with dyspnoea on exertion

We present the case of a 56-year-old woman suffering from shortness of breath on exertion (NYHA class II) worsening for the last couple of years. Blood pressure on admission was 140/71 mm Hg and heart rate (HR) was 72 bpm. Further physical examination and routine laboratory tests were irrelevant. The electrocardiogram showed normal sinus rhythm (SR) with HR 62 bpm, intraventricular conduction delay, a low progression of R wave in V1–V4 leads and premature ventricular contractions. 24-hour Holter electrocardiogram monitoring was carried out. Normal SR with a mean HR of 67 bpm was recorded. There were 967 premature ventricular complexes (PVCs), including one run of three PVCs, two pairs of PVCs, 27 bigeminies and nine trigeminies. Moderate mitral regurgitation, akinesis of the apex, posterior and inferior walls and asynchrony of the septum contraction were diagnosed with echocardiography. The ejection fraction was 45%. Colour Doppler visualised the collaterals connecting the right coronary artery (RCA) and the left coronary artery (LCA). The last one appeared to conjoin with the pulmonary trunk. Coronarography confirmed the presence of wide RCA which connected with the branches of LCA. The origin of LCA could not be visualised in the ascending aorta. The coronary arteries anomaly was confirmed in angio-computed tomography (CT), which showed that left main coronary artery arises from the left-inferior part of the pulmonary trunk (Fig. 1). The left anterior descending artery and left circumflexus were patent, and sinuous with a diameter of 8 mm. RCA was dominant, very sinuous, and originating from the typical place. The junctions between distal branches of RCA and LCA were observed (Fig. 2). Magnetic resonance imaging of the heart revealed dilation of the left ventricle which was generally hypokinetic. Late gadolinium enhancement was not observed in the left ventricle muscle. A diagnosis of ALCAPA syndrome, anomalous origin of the LCA from the pulmonary artery, also called Bland-White-Garland syndrome, was established. This rare congenital anomaly appears in 1 in 300,000 live births and accounts for less than 0.5% of all congenital heart anomalies. In most cases, diagnosis and treatment is carried out in the first year of life. It is a very rare situation when ALCAPA is diagnosed in an adult because the natural history of this malformation mostly leads to death in childhood. There is a consensus that children with ALCAPA will benefit from a replantation of LCA to the ascending aorta. This procedure is technically impossible in most adults. Thus LCA bypass grafting with subsequent ligation of its anomalous origin should be performed. Another surgical method is the Takeuchi procedure where an intrapulmonary tunnel joining LCA and the left aortic sinus is created. Even asymptomatic patients should undergo surgery to prevent sudden cardiac death. Our patient was fully informed about the possible options but declined the surgical therapy. She was prescribed the following medications: carvedilol, perindopril, spironolactone, indapamide, rosuvastatin and acetylsalicylic acid, and returned home.

Endovascular Treatment of Late Thoracic Aortic Aneurysms after Surgical Repair of Congenital Aortic Coarctation in Childhood

Background In some patients, local surgery-related complications are diagnosed many years after surgery for aortic coarctation. The purposes of this study were: (1) to systematically evaluate asymptomatic adults after Dacron patch repair in childhood, (2) to estimate the formation rate of secondary thoracic aortic aneurysms (TAAs) and (3) to assess outcomes after intravascular treatment for TAAs. Methods This study involved 37 asymptomatic patients (26 female and 11 male) who underwent surgical repair of aortic coarctation in the childhood. After they had reached adolescence, patients with secondary TAAs were referred to endovascular repair. Results Follow-up studies revealed TAA in seven cases (19%) (including six with the gothic type of the aortic arch) and mild recoarctation in other six (16%). Six of the TAA patients were treated with stentgrafts, but one refused to undergo an endovascular procedure. In three cases, stengrafts covered the left subclavian artery (LSA), in another the graft was implanted distally to the LSA. In two individuals, elective hybrid procedures were performed with surgical bypass to the supraaortic arteries followed by stengraft implantation. All subjects survived the secondary procedures. One patient developed type Ia endoleak after stentgraft implantation that was eventually treated with a debranching procedure. Conclusions The long-term course of clinically asymptomatic patients after coarctation patch repair is not uncommonly complicated by formation of TAAs (particularly in individuals with the gothic pattern of the aortic arch) that can be treated effectively with stentgrafts. However, in some patients hybrid procedures may be necessary.