Left coronary arteriovenous malformation with fistulous connections to the left and right ventricles.

A 20-year-old man with right bundle branch block in recorded ECG was referred to our department. His physical examination was unremarkable. Transthoracic echocardiography showed a severe hypertrophy of the interventricular septum (22 mm)which contained multiple echo-free spaces of the vascular nature. A flow pattern suggestive to a coronary artery fistula into the left ventricle was recorded. The patient was referred for a coronary angiography, which revealed an arteriovenous malformation starting from the septal branch of the enlarged left anterior descending artery. The malformation communicated with the lumen of the left (arterial phase) and right ventricle(venous phase) as well. The posterior descending artery was fed exclusively from the described arteriovenous malformation.99mTc MIBI SPECT images showed a moderately reversible perfusion defect in the inferior wall, suggesting non-critical ischemia of this region.

Heart structure and function in patients with generalized autoimmune diseases: echocardiography with tissue Doppler study.

OBJECTIVE: Heart pathology strongly influences the course and prognosis of patients with generalized autoimmune diseases. In spite of autoimmunity being a common denominator of these diseases, systemic sclerosis (SSc), systemic lupus erythematosus (SLE) and dermato/polymyositis (DPM) differ significantly in the pathogenesis of organ damage. The aim of the study was to compare pathologic changes in heart structure and function in these diseases by means of standard echocardiography and tissue Doppler (TDE). MATERIAL AND METHODS: Four groups were examined: 60 SSc, 60 SLE and 15 DPM patients in stable clinical conditions and 30 healthy control subjects. Echocardiography with TDE was performed with the assessment of systolic (S) and diastolic (E) velocities of mitral and tricuspid annuli. RESULTS: Heart in SSc was characterized by significant diastolic left ventricular dysfunction (mitral E 8.61 +/- 2.3 cm/s vs. 12.4 +/- 3.5 cm/s in the control group; P < 0.01) with preserved systolic function (mitral S 7.85 +/- 1.5 cm/s vs. 7.95 +/- 0.9 cm/s in control group; ns). SLE and DPM resulted mainly in pathologic thickening of valvular leaflets and/or pericardium [mitral or aortic leaflets thickened in 38 (63.3%) of SLE patients, 7 (46.7%) of DPM patients; pericardium thickened in 36 (60%) of SLE patients]. Pulmonary capillary wedge pressure was elevated in SSc (13.8 +/- 3.5 mmHg) and DPM (13.2 +/- 2.5 mmHg) patients as compared to the control group (9.2 +/- 3.7 mmHg, P < 0.01). Right ventricular systolic and diastolic dysfunction was frequent irrespective of the presence or absence of pulmonary hypertension. CONCLUSIONS: Echocardiography with TDE reveals characteristic pathology in different forms of generalized autoimmune diseases reflecting their different pathogenetic mechanisms. Overproduction of collagen in SSc results in diastolic left ventricular dysfunction, while generalized inflammation in SLE and DPM leads mainly to pathologic changes on valvular leaflets and/or pericardium. Interestingly, right ventricular dysfunction is common in all diseases analyzed, regardless of the presence of pulmonary hypertension. Echocardiography, preferably with TDE, could add valuable information about usually asymptomatic heart pathology in an individual patient with generalized autoimmune disease.

Diastolic heart dysfunction, increased pulmonary capillary wedge pressure and impaired exercise tolerance in patients with systemic sclerosis.

BACKGROUND: Diastolic heart dysfunction, responsible for dyspnoea in heart failure patients, is an important prognostic factor. Patients with systemic sclerosis (SSc) serve as a model of diastolic heart failure with preserved ejection fraction. AIM: To quantify diastolic left ventricular (LV) dysfunction and elevation of pulmonary capillary wedge pressures (PCWP) in SSc patients and to assess the effects of these parameters on exercise tolerance. METHODS: In 46 SSc patients (43 females, three males, aged 24-73 years) and 30 healthy females, echocardiography with tissue Doppler (TDE) and cardiopulmonary exercise tests (CPX) were performed. During TDE, the systolic (S) and early diastolic (E) velocities of mitral annulus were recorded. The PCWP was calculated on the basis of mitral inflow E velocity and E velocity of mitral annulus. The CPX was performed using a modified Bruce protocol. RESULTS: Left ventricular ejection fraction was normal in the SSc group. Mitral inflow E/A ratio was pseudonormal in five SSc patients, and significantly decreased in the remainder as compared to controls (0.87 ± 0.2 vs 1.38 ± 0.5, p < 0.0002). The TDE examination confirmed normal systolic LV function, but severe LV diastolic dysfunction (E 8.66 ± 2.5 cm/s vs 12.39 ± 3.5 cm/s in controls, p < 0.000002). The PCWP was higher in the SSc group (11.8 ± 3.3 mm Hg vs 7.7 ± 1.7 mm Hg in controls, p < 0.0001). The PCWP > 10 mm Hg significantly decreased exercise duration, maximal oxygen uptake and carbon dioxide output and identified patients with oxygen uptake < 20 mL/kg/min with 100% sensitivity and 78% specificity. The ventilatory equivalent of carbon dioxide was increased in the SSc group (VE/VCO2 38.7 ± 7.5 vs 30.55 ± 4.2 in controls, p < 0.002). CONCLUSIONS: Pure LV diastolic dysfunction, typical of SSc, leads to the elevation of PCWP. Values of PCWP > 10 mm Hg are associated with severe exercise intolerance demonstrated by shorter duration of exercise with decreased oxygen uptake and carbon dioxide output during exercise.

Transcatheter closure as an alternative and equivalent method to the surgical treatment of atrial septal defect in adults: comparison of early and late results.

BACKGROUND: The clinical efficacy of transcatheter atrial septal defect (ASD) closure with the Amplatzer Septal Occluder (ASO) can only be judged against the results of contemporaneous surgery. The present study compared early and late results of ASD closure using ASO versus open-heart surgery. METHODS: Forty-eight adult patients were found eligible for transcatheter closure in transesophageal echocardiography. The surgical group consisted of 52 patients with isolated ostium secundum ASD. All patients underwent standard ECG, 24-hour ECG recording, and transthoracic echocardiography pre-procedure at 1-month and at 1-year follow-up. Physical fitness was assessed by cardiopulmonary exercise testing (CPX) prior to ASD closure and at 1-year follow-up. RESULTS: ASD closure was successful in all surgical patients and in 94% of the ASO group (0% mortality). The total complication rate for surgical vs. device closure was not significantly different (19.2% vs. 26.7%; p=0.383), despite more serious complications in the surgical group. Hospital stay was significantly shorter in the ASO group (5.4+/-2.2 vs. 9.1+/-1.2 days; p<0.001). Although echocardiographic parameters did not differ significantly between the respective groups at 1-year follow-up, CPX revealed a higher decrease in the VE/VCO2 slope in the ASO group (-3.7+/-3.4 vs.-1.2+/-4.8; p=0.003). CONCLUSIONS: As surgical and device closure appear similarly effective in adults with ASD, avoidance of thoracotomy and cardiopulmonary bypass, in conjunction with a shorter hospital stay, argues in favour of device closure in selected patients.

[Cardiopulmonary capacity and hemodynamic changes in adults with atrial septal defect]. / Ocena wydolnosci wysilkowej i zmian hemodynamicznych u doroslych z ubytkiem przegrody miedzyprzedsionkowej.

Little is known about the relation between echocardiographic parameters and cardiopulmonary capacity in patients with atrial septal defect (ASD). The study aimed to assess the value of right atrial, right ventricle and left ventricle dimensions and function in predicting cardiopulmonary capacity in adults with ASD. 52 consecutive adult patients with hemodynamic significant ASD (mean age 38.6+/-15, range: 18-68 years; mean Qp/Qs: 2.7+/-0.7, range: 1.5-4.3) were enrolled into the study. The right ventricle indices were defined as follows: end-diastolic and end-systolic area, ejection fraction (area-length method) and right ventricle systolic pressure (RVSP). The following right atrial parameters were taken into consideration: end-diastolic and end-systolic right atrial area. Left ventricular systolic and diastolic diameters and LV ejection fraction were measured as well. All patients underwent the cardiopulmonary exercise test (CPX) with estimation of peak oxygen uptake -VO2, anaerobic threshold -AT and VE/VCO2. The mean VO2 was 23.4+/-18.4 ml/kg/min (63+/-18.4% of the predicted value). The dimensions and ejection fraction of left ventricle did not correlate significantly with any CPX parameter. Echocardiographic parameters of both right atrial and right ventricle function were related to peak oxygen uptake in adults with ASD. The strongest correlation between VO2peak and RVSP was found (r=-0.7; p<0.001). Echocardiography seems to be efficient in predicting cardiopulmonary capacity in ASD patients.

[Evaluation of left ventricular function in patients with chronic obstructive pulmonary disease]. / Ocena czynnosci lewej komory u chorych na przewlekla obturacyjna chorobe pluc.

INTRODUCTION: Patients with right ventricle (RV) pressure overload often have impaired left ventricular (LV) diastolic function. Objectives. The aim of study was to evaluate LV function in patients with chronic obstructive pulmonary disease (COPD). Patients and methods. Thirty-five patients (mean age: 62.1 +/- 7.7 y) with COPD without additional cardiac diseases and 25 age--and sex-matched healthy subjects were enrolled into the study. All patients underwent resting ECG tracing, blood pressure, spirometry, standard and tissue Doppler echocardiography. RESULTS: The mean value of forced expiratory volume in one second (FEV1) in the COPD group was 40 +/- 8.9% of the predicted value. We found no significant differences in LV end-diastolic and systolic diameter and interventricular septum as well between COPD patients and controls. RV end-diastolic diameter and RV wall thickness were significantly larger and right ventricle systolic pressure--RVSP (38 +/- 11.2 vs. 20 +/- 2.5 mm Hg) significantly higher in the COPD group. Both peak early to peak atrial filling velocities ratio--E/A and peak annular velocity during early diastole to peak annular velocity during atrial contraction--Em/Am were significantly lower in COPD compared to controls. Moreover, there was a strong inverse correlation between Em/Am and RVSP (r = -0.75; p < 0.001) and between E/A (r = -0.6; p < 0.001) as well. We found no significant differences in parameters assessing the LV systolic function between both groups. CONCLUSIONS: In COPD patients LV diastolic function is significantly impaired and its magnitude is related with increase in pulmonary artery pressure, while systolic LV function is well preserved.

Atrial septal defect in adults: the influence of age and haemodynamic parameters on the results of surgical repair.

INTRODUCTION: Indications for closure of atrial septal defect (ASD) are well established in children but still unclear in adult patients. There is also a discrepancy regarding the natural history of the defect and benefits of ASD closure and long-term prognosis. AIM: The aim of the study was to evaluate the outcome of surgical closure of atrial septal defect (ASD) with respect to age and selected haemodynamic parameters. METHODS: The study involved 52 patients with ASD, aged 38.6+/-15 years. All patients had a clinical examination, echocardiography, Holter ECG monitoring and cardiopulmonary exercise test (CPX) prior to and at least one year after the surgery. The results were analysed for the entire group of patients and then for the following subgroups of pts.: aged <40 and l40 years; with right ventricular systolic blood pressure (RVSP) < or =30 and >30 mmHg; and with shunt volume ratio (Qp/Qs) <2.5 and > or =2.5. RESULTS: After repair of ASD, a significant improvement of the clinical status, as defined by NYHA class, was observed. At the same time, a constant or even increased incidence of arrhythmias was found. Repair of the defect resulted in a significant reduction of the right ventricular diameters in all studied subgroups. Among all evaluated parameters, only age at time of the operation was significantly associated with right ventricular diameter after surgery (b=0.598; p <0.001). Exercise capacity was found to have significantly increased after surgery, regardless of age, RVSP and Qp/Qs, but it remained significantly reduced in patients with pulmonary arterial hypertension compared to subjects with normal RVSP before ASD repair. A significant correlation was shown between peak oxygen consumption and RVSP both before (r=-0.68; p <0.001) and after (r=-0.38; p=0.01) surgery. CONCLUSIONS: Patients benefit from surgical closure of ASD regardless of age and previous RVSP and Qp/Qs. Age at the time of surgery and pulmonary arterial pressure determine long-term results and improvement of echocardiographic and ETT parameters.

Atrial septal defect in adults: echocardiography and cardiopulmonary exercise capacity associated with hemodynamics before and after surgical closure.

The study aimed to evaluate pre and postoperative echocardiographic data and exercise capacity in relation to age and hemodynamics in adults with atrial septal defect (ASD). Fifty-two subjects with ASD (mean age: 38.6+/-15 years) were enrolled. Echocardiography and cardiopulmonary exercise test were performed before and a year after surgery. Pre and postoperative data were analyzed for the entire group and then compared in terms of age: <40 and >or=40 years, right ventricular systolic pressure (RVSP): 30 mmHg and pulmonary to systemic flow ratio (Qp/Qs): <2.5 and >or=2.5. After surgery right ventricle dimension decreased in all patients, although it remained significantly larger in patients over 40 years. There was a negative correlation between peak oxygen uptake and preoperative RVSP (r=-0.69, P<0.001). Exercise capacity improved irrespective of the age at surgery, preoperative RVSP and Qp/Qs, although it failed to normalize in patients with RVSP >30 mmHg. Adults with ASD benefit from surgical closure irrespective of the actual age at surgery. Patient's age at surgery and pulmonary hypertension crucially impact the results of surgical intervention. Early defect correction is therefore highly recommendable, specifically with a view to preventing the hemodynamic consequences of ASD.

[Early results of atrial septal defect closure with the Amplatzer Septal Occluder are impacted by tricuspid insufficiency]. / Przezskórne zamykanie ubytków w przegrodzie miedzyprzedsionkowej przy pomocy zapinki Amplatza: wczesne rezultaty w zaleznosci od niedomykalnosci trójdzielnej.

OBJECTIVE: Our study reports the results of a comparison of transcatheter closure of secundum atrial septal defect (ASD) using the Amplatzer Septal Occluder (ASO) in patients with and without tricuspid insufficiency. METHODS: Consecutive 27 adult patients (20 females, 7 males) with a mean age of 41.1 +/- 13.3 (range 18-62) years having an ASD II and a transcatheter closure between December 2000 and December 2002 were analyzed. All the patients had an isolated secundum ASD with a significant left-to-right shunt (ratio of pulmonary to systemic blood flow or Qp:Qs >1.5:1). Patients were divided in two groups: group I--without tricuspid abnormalities (8 patients, 29.6%), group II--patients with tricuspid insufficiency I-III stage, (19 patients, 70.4%). Transthoracic color Doppler echocardiographic study was performed in all patients before discharge and was repeated one month after discharge. RESULTS: The mean age, the diameter of defect, the degree of left-to-right shunt, the diameter of implanted device were comparable in both groups. Before implantation the dimension of right heart cavities differed between groups. The right ventricular dimension was larger in group II (mean 36.2 mm vs. 29.1 mm; p<0.009), the right atrium diameter was larger in group II (46.2 mm vs. 24.3 mm; p<0.007). Pulmonary artery systolic pressure before the procedure was higher in group II (36.6 mmHg vs. 20.43 mmHg; p<0.006). The ASO device was successfully implanted in all the patients. At one month follow-up septal motion abnormalities normalized in all patients in both groups. The mean decrease of right atrium diameter in group I was 6.2 mm (range 1.5-12 mm) compared to 9.6 mm (range 2-20) in group II, (p<0.001), the mean decrease of right ventricular diameter in group I was 5.8 mm (range 2-8 mm) compared to 3.1 mm (range 1-5.9) in group II, (p<0.006). CONCLUSIONS: Short-term follow-up demonstrated excellent results of ASD closure in both groups. In one month follow-up the decrease of right cavities dimensions was significantly higher in the patients without tricuspid abnormalities. The pulmonary artery systolic pressure before ASD closure was significantly higher in the patients with tricuspid abnormality.

[Left ventricle TEI index--assessment of clinical role in patients with aortic valve stenosis]. / Ocena przydatnosci indeksu TEI u chorych ze zwezeniem zastawki aortalnej.

AIM: The study aimed to assess the clinical efficacy of the TEI index in patients with aortic valve stenosis (AVS). METHODS: The study group comprised 30 consecutive patients (12 F; 18 M; mean age: 60.1 +/- 9.2; range: 48-70 years) with aortic valve stenosis. Their clinical status was evaluated according to the NYHA criteria. A complete 2-dimensional, Doppler and color flow Doppler examination was performed. To assess systolic and diastolic left ventricle function we used ejection fraction--EF (%) and transmitral inflow velocity E/A ratio, respectively. The TEI index was calculated as (a - b)/b, were a is the interval between the cessation and onset of mitral inflow, and b is the ejection time. We also determined: maximal transaortic gradient--Ao max gr (mmHg), aortic valve area--AVA (cm2) and left ventricular mass index--LV mass index (g/m2). Based on clinical symptoms we divided patients into two groups: Group 1 (NYHA I/II) and Group 2 (NYHA III and IV). RESULTS: There were no significant differences in age, Ao max gr, AVA, EF and ejection time (b) between groups. But we found significantly prolonged time interval (a) and consequently significantly higher TEI index (p<0.001) in the group with severe symptoms. LV mass index and E/A ratio were also related to the patient's clinical status (p<0.05) but the TEI index seems to be more sensitive in distinguishing severely limited patients. CONCLUSION: The TEI index revealed to be closely related to symptoms in patients with aortic valve stenosis. The patient's clinical status seems to depend more on the TEI than on the conventional measured parameters like aortic valve area and transaortic gradient.

[Atrial septal defect associated with mitral valve prolapse--prevalence and clinical significance]. / Ubytek przegrody miedzyprzedsionkowej skojarzony z wypadaniem platków zastawki mitralnej--czestosc wystepowania i znaczenie kliniczne.

AIM: High incidence of mitral valve prolapse (MVP) associated with atrial septal defect (ASD) has been reported. The study aimed to evaluate the prevalence, etiology and clinical significance of MVP in patients with ASD. METHODS: Forty-seven consecutive patients with secundum type ASD (30 F; 17 M; mean age: 37.9 +/- 14.0; range: 16-62 years) were enrolled into the study. All patients underwent M-mode and two dimensional echocardiography to check for MVP, defined as the superior systolic displacement of mitral leaflets > or = 2 mm above annulus with coaptation point at, or superior to the annular plane. Pulmonary to systemic flow ratio (Qp/Qs), diastolic right ventricle dimension (RV), left ventricle dimension (LV) and left to right ventricle ratio (LV/RV) were measured. Furthermore, mitral and tricuspid valve insufficiency and right ventricle systolic pressure (RVSP) were evaluated. A symptom-limited, incremental exercise test (CPX)--modo Bruce on Marquette 2000 Treadmill was performed in every patient. We determined: time of exercise--Time (min), peak oxygen uptake--VO2peak (ml/kg/min), VO2peak expressed as % of predicted value--VO2% and anaerobic threshold--AT (expressed as % VO2 max). The study population was divided into two groups: Group I--patients with ASD and MVP and Group II--patients with ASD without MVP. RESULTS: MVP was recognized in 17 patients (36%); anterior MVP was found in 14, two cases revealed posterior MVP and one was diagnosed with both anterior and posterior MVP. MVP was not associated with significant mitral regurgitation. Echocardiographic and CPX data are summarized in table I. There were no significant differences in age, RVSP, RV and LV diastolic dimensions between groups, although Qp/Qs was significantly higher (p=0.01) and LV/RV significantly lower (p=0.02) in the MVP group. Moreover, there was a significant negative correlation between Qp/Qs and LV/RV ratio (r=-0.70; p<0.001) in a study group. No significant difference in time of exercise, VO2peak, VO2%, and AT was observed between respective groups. CONCLUSIONS: Our data support the thesis that MVP associated with secundum ASD is a functional disorder due to the atrial shunt and leftward shift of interventricular septum. MVP does not affect cardiopulmonary capacity in ASD patients.

[Cardiopulmonary exercise capacity in adult patients with atrial septal defect]. / Ocena wydolnosci wysilkowej doroslych chorych z ubytkiem w przegrodzie miedzyprzedsionkowej.

UNLABELLED: The aim of the study was to evaluate cardiopulmonary exercise capacity (WTS) in adult patients with atrial septal defect (ASD). After excluding 10 patients with additional cardiac, pulmonary or muscle diseases, 53 patients with ASD (Gr-ASD; mean age 37.8 +/- 14.9 years; 35 women, 19 men) were enrolled in our study. In addition, we evaluated 22 healthy age and gender matched control subjects (Gr-K; mean age 36.6 +/- 14.9 years; 13 women, 9 men). First, all subjects underwent resting spirometry and forced vital capacity--(FVC; % of predicted value), one-second forced expiratory volume--(FEV1; % of predicted value) and FEV1/FVC (%) were determined. Then each subject performed a symptom-limited, incremental exercise test (modo Bruce). We evaluated the following parameters of resting metabolism: respiratory rate--(RR; L/min), minute ventilation--(VE; L/min), tidal volume--(Vt; L), oxygen uptake--(VO2; ml/kg/min), end-tidal carbon dioxide pressure--(PET CO2; mmHg), end tidal oxygen pressure--(PET O2; mmHg), ventilatory equivalent for carbon dioxide--(VE/VCO2) and ventilatory equivalent for oxygen--(VE/VO2). The following exercise parameters were analysed: peak oxygen uptake--(VO2 peak; ml/kg/min), VO2 peak expressed as % of predicted value--(VO2 %N), anaerobic threshold--(AT; % VO2 max), ventilatory equivalent for carbon dioxide--(VE/VCO2), end-tidal carbon dioxide pressure--(PET CO2; mmHg), O2 pulse, time of exercise--(T; min) and time to AT--(TAT; min). In addition, we performed a subgroup analysis for ASD patients below and > or = 40 years of age. All values were expressed as mean +/- SD. RESULTS: Although there was no significant difference in FVC, FEV1, FEV1/FVC, VO2, VE, Vt, RR between ASD patients and controls, the ASD patients aged 40 or older showed significantly lower FEV1/FVC and significantly higher RR. Expiratory gas exchange analysis showed significant differences between the ASD patients and controls (higher VE/VCO2, VE/VO2, PET CO2, and lower PET O2 respectively). Moreover, VO2 peak, VO2%N, AT, PET CO2 and O2 pulse were significantly lower while VE/VCO2 was significantly higher in ASD patients than in normal subjects. On average, VO2%N in ASD patients was only 61% of the predicted value for age and sex. Total time of exercise and time to AT were significantly shorter in ASD patients as well. CONCLUSIONS: WTS allows appropriate evaluation of cardiopulmonary capacity in ASD patients, though the mechanisms underlying ventilatory and hemodynamic abnormalities are still not fully understood. Adult patients with ASD reveal ventilatory abnormalities with an age-related trend towards deterioration. Cardiopulmonary exercise capacity in adults with ASD is markedly reduced in comparison with healthy population and deteriorates with age. WTS complements echocardiographic and hemodynamic evaluation of patients with ASD, and is helpful in indicating patients for ASD closure and their follow up.