Ayşe Güler Eroğlu

Evolution of ventricular septal defect with special reference to spontaneous closure rate, subaortic ridge and aortic valve prolapse.

The medical records of 685 patients with ventricular septal defect (VSD) were reviewed to determine spontaneous closure, left ventricular-to-right atrial shunt, subaortic ridge, and aortic valve prolapse. Patients had been followed for a mean of 3 ± 2.5 years and median 2.2 years by echocardiography. VSD was perimembranous in 65.7% (450), trabecular muscular in 30.8% (211), muscular outlet in 2.3% (16), muscular inlet in 0.7% (5), and doubly committed subarterial in 0.5% (3). Defect size was classified in 76% (517) as small, in 18% (124) as moderate, and in 6% (44) as large. VSD closed spontaneously in 27% (186 of 685 patients) by ages 40 days to 13.7 years (mean, 2.1 ± 2.2 years; median, 1.3 years). Sixty-six of 450 perimembranous defects (15%) and 120 of 211 trabecular muscular defects (57%) closed spontaneously (p <0.001). Defect size became small in 15% of patients with VSD at mean 2.9 ± 2.3 years (median, 2.3 years). Aneurysmal transformation was detected in 56% (254), left ventricular-to-right atrial shunt in 8.4% (38), subaortic ridge in 5.8% (26), aortic valve prolapse in 11.7% (53), and aortic regurgitation in 7.3% (33) of 450 patients with perimembranous defect. There was no statistical significance between the age at closure and the age of detection of aneurysmal transformation in the patients with perimembranous defect (p = 0.25).

Aortic valve prolapse and aortic regurgitation in patients with ventricular septal defect.

Aortic valve prolapse (AVP) was detected in 82 (7.5%) of 1096 patients with ventricular septal defect (VSD) (in 50 at initial echocardiographic examination and in 32 at follow-up) by echocardiography. Of 82 patients with AVP, aortic regurgitation (AR) was detected in 53 (65%) (in 28 at initial echocardiographic examination and in 25 at follow-up), resulting in an incidence of AR of 4.8% (of VSD). The percentage of AVP (20.8%) and AR (16.7%) in muscular outlet VSDs was larger than the percentage of AVP (10.6%) and AR (6.8%) in perimembranous VSDs (p <0.05). Fourty-four patients were followed medically after AVP appeared (3 months to 10.8 years; median, 2.1 years). Initially, there was no AR in 24 of these patients, trivial AR in 7, and mild AR in 13. Trivial AR developed in 6 (25%) and mild AR developed in 3 (13%) of 24 patients who had no AR (in 5 of them within 1 year and in 9 of them within 2 years). In 2 (29%) of 7 patients, trivial AR progressed to mild AR during a median of 2 years, and in 4 (31%) of 13 patients, mild AR progressed to moderate AR during a median of 1.1 years. We recommend frequent echocardiographic evaluation (every 6 months) for detecting of appearance of AR in patients with perimembranous or muscular outlet VSD after AVP develops and for evaluating the progression of AR in patients with perimembranous or muscular outlet VSD, AVP, and trivial AR. In addition, we recommend surgical intervention in patients with perimembranous or muscular outlet VSD, AVP, and mild AR because of rapid progression of mild AR to moderate AR.

Echocardiographic Follow-Up of Children with Isolated Discrete Subaortic Stenosis

This study evaluates the progression of stenosis, onset and progression of aortic regurgitation (AR), and the results of surgical outcomes in children with isolated discrete subaortic stenosis (SAS). The medical records of 108 patients (mean age, 5.5 ± 3.8 years; range, 3 days to 18 years) with isolated discrete SAS were reviewed. Patients with lesions other than AR were excluded. Very mild stenosis was defined as Doppler peak systolic instantaneous gradient (PSIG) less than 25 mmHg, mild stenosis as 25–49 mmHg, moderate stenosis as 50–75 mmHg, and severe stenosis as more than 75 mmHg. Seventy-eight of 108 patients were followed for 2 months to 14 years (mean, 4.8 ± 3.7 years; median, 5 years) with medical treatment alone. In these patients, the mean PSIG at last echocardiogram was higher than the mean PSIG at initial echocardiogram (39 ± 19 vs 31 ± 12 mmHg, respectively; p < 0.001). Among 24 patients with very mild stenosis at initial echocardiogram, 10 had mild and 2 had moderate stenosis after a mean period of 5.6 years. Among 46 patients with mild stenosis at initial echocardiogram, 11 had moderate and 5 had severe stenosis after a mean period of 4.1 years. Only 1 patient among the 8 patients with moderate stenosis at initial echocardiogram had severe stenosis after a mean period of 2.7 years. Thirty-nine patients (50%) had AR (13% trivial, 33% mild, and 4% moderate) at initial echocardiogram. After a mean period of 4.8 years, 77% of the patients had AR (10% trivial, 53% mild, 9% mild–moderate, and 5% moderate). Twenty-four patients underwent surgery. Preoperatively, mean Doppler PSIG and AR incidence were 64 ± 17 mmHg and 91% (22/24), respectively. The mean Doppler PSIG was 30 ± 19 mmHg and AR was present in all of the patients a mean period of 4.1 years after surgery. Two patients underwent reoperation for recurrent SAS and AR. Patients with very mild or mild stenosis may be followed noninvasively every year. One patient of the 8 patients with moderate stenosis progressed to severe stenosis, and moderate AR developed in 2 patients after a mean of 2.7 years. We recommend that patients with moderate stenosis undergo careful evaluation to determine whether surgery is necessary due to the severity of stenosis and AR.

Anomalous origin of circumflex coronary artery from the right pulmonary artery associated with subaortic stenosis and coarctation of the aorta.

Anomalous origin of the circumflex coronary artery is extremely rare and may cause acute cardiac decompensation associated with correction of coexisting congenital malformations. We describe a 10-year-old female patient who underwent surgical repair of the aortic coarctation at 4 years of age. Six years later, she presented with chest pain during exercise. Cardiac catheterization demonstrated 25 mmHg subaortic systolic gradient and retrograde filling of the circumflex coronary artery from the left anterior descending and right coronary artery, with drainage into the right pulmonary artery. Reimplantation of the anomalous circumflex coronary artery to the aorta and resection of subaortic fibrous membrane was performed. Her postoperative course was uneventful, with complete relief of symptoms.

Outcome for patients with isolated atrial septal defects in the oval fossa diagnosed in infancy

OBJECTIVE Our aim was to investigate the change in diameter of holes within the oval fossa, and the role of aneurismal formation in reducing the size of the hole, in patients diagnosed during infancy with isolated defects in the floor of the fossa. PATIENTS In a retrospective study, we included 100 patients diagnosed during the first year of life with an isolated defect in the floor of the oval fossa who had subsequently been observed for at least 5 years. There were 56 females and 44 males. They had been admitted to hospital because of a murmur in 65, heart failure in 9, and other reasons in 17. The remaining 9 patients were referred from other institutions with an established diagnosis of defects within the oval fossa. Patients were grouped according to the size of the deficiency in the floor of the fossa. Defects of diameter less than 5 mm were considered to be small, and 20 patients had such defects. Medium sized defects were judged to be between 5 and 8 mm, with 26 patients fulfilling this criterion, with the other 54 patients having large defects with diameters greater than 8 mm. RESULTS The overall spontaneous rate of closure was 27%. Of those with medium defects, half closed spontaneously, but only 7.5% of those with large defects showed such closure. Of the patients who were diagnosed with heart failure, 9 had defects measuring 7 mm, and of these, 6 required surgical closure, 1 patient had spontaneous closure, while the defect became smaller in the remaining 2. On the other hand, in 9 out of 10 patients who had aneurysms, the diameter of the defect was between 7 and 15 mm. Amongst these patients, the defect closed spontaneously in 3, and reduced in size in the others. CONCLUSION When holes within the oval fossa measure 8 mm or below, the majority of patients with experience either spontaneous closure or show decrease in size of the defect. In those with larger defects, the size usually increases, and surgery is needed for closure. If there is aneurismal formation, however, even when the defect measures more than 8 mm, the defect usually closes on its own or gets smaller.

Patency or recanalization of the arterial duct after surgical double ligation and transfixion.

OBJECTIVE The frequency of residual shunting or recanalization was investigated in patients in whom a persistently patent arterial duct had been doubly ligated and transfixed during surgical closure. METHODS We investigated in retrospective fashion for any residual shunting 325 patients who, between January 1990 and December 2004, had undergone surgical double ligation and transfixion of a persistently patent arterial duct. Shunting was discovered in 10 patients, of whom four male and six female. RESULTS Of those with residual shunting. 4 patients had initially exhibited only persistent patency of the duct, while the other 6 had associated mild cardiac lesions. The mean age at operation was 5.5 years, with a range from 0.5 to 17.9 years. Postoperatively, the mean period for detecting the residual shunt was 22.8 months, with a range from 2 days to 72 months. The frequency of residual shunting amongst our patients, therefore, was 3.1%. We detected the residual shunt by colour-flow Doppler mapping in all patients, although a continuous murmur was heard in only one patient on physical examination. CONCLUSION Our findings suggest that clinical sensitivity of detecting residual shunting subsequent to surgical closure of the persistently patent arterial duct is low, and hence that colour-flow Doppler interrogation should be a part of follow up. Residual shunting, or recanalization, may occur even after double ligation and transfixion of the duct. Since the residual flow may emerge after months, or even years, follow-up is needed for longer periods.

Echocardiographic follow-up of congenital aortic valvular stenosis.

We investigated the morphology of the stenotic aortic valve, the progression of the stenosis, and the onset and progression of aortic regurgitation (AR) in patients with congenital aortic valvular stenosis (AVS). The medical records of 278 patients with AVS were reviewed, with the patients with concomitant lesions besides AR excluded. Very mild aortic stenosis was defined as a transvalvular Doppler peak systolic instantaneous gradient (PSIG) less than 25 mmHg, mild stenosis as 25–49 mmHg, moderate stenosis as 50–75 mmHg, and severe stenosis as more than 75 mmHg. The mean age of the patients was 4.9 ± 4.3 years (range, 3 days to 15 years), and 203 (73%) were male. The number of the cusps was determined with two-dimensional echocardiography in 266 patients (95%): unicuspid in 3 patients (1%), bicuspid in 127 patients (48%), and tricuspid in 136 patients (51%). A total of 192 of all patients were followed for 2 months to 14.6 years (mean 4.2 ± 3.3 years) with medical treatment alone. Among 72 patients with very mild stenosis at initial echocardiographic examination, 20% had mild, 3% moderate, and 1% severe stenosis after a mean period of 3.7 years. In 70 patients with mild stenosis at initial echocardiographic examination, 28% had moderate and 9% severe stenosis after a mean period of 5 years. Among 44 patients with moderate stenosis at initial echocardiographic examination, 36% had severe stenosis after a mean period of 3.7 years. Among 192 patients, 40% had AR (3% trivial, 28% mild, and 9% moderate) at initial echocardiographic examination. After a mean period of 4.2 years, 58% of the patients had AR (13 % trivial, 25% mild, 16% moderate, and 4% severe). There was not statistically significant difference between catheterization peak systolic gradients (47 ± 16 mmHg) and Doppler estimated mean gradients (45 ± 9 mmHg) (p = 0.53), whereas Doppler PSIGs (74.9 ± 15.7 mmHg) were higher than catheterization peak systolic gradients (p < 0.0001) in 25 patients who were studied in the catheterization lab. Patients with very mild stenosis may be followed with a noninvasive approach every 1 or 2 years, and an annual follow-up is suggested for patients with mild stenosis. Nearly one-third of patients with moderate stenosis at initial echocardiographic examination had severe stenosis after a mean period of 3.7 years. Therefore, we recommend, that patients with moderate stenosis undergo noninvasive evaluation every 6 months. Doppler estimated mean gradient is very useful in predicting the need for intervention in children with AVS.

QT dispersion and cardiac involvement in children with Familial Mediterranean fever

Familial Mediterranean fever is a hereditary disease characterised by recurrent and self-terminated attacks of fever and polyserositis. An earlier study found that adult patients of Familial Mediterranean fever had an abnormally longer QT dispersion and corrected QT dispersion, markers for ventricular arrhythmogenicity. QT dispersion is a simple non-invasive arrhythmogenic marker that can be used to assess homogeneity of cardiac repolarisation; however, it has not been studied in children with Familial Mediterranean fever before. The aim of this study was to assess QT dispersion and corrected QT dispersion, and their relationship with systolic and diastolic function of the left ventricle in a group of children with Familial Mediterranean fever. We performed electrocardiography and Doppler echocardiography on patients and controls. Maximum QT, minimum QT, QT dispersion, corrected QT, maximum corrected QT, minimum corrected QT, and corrected QT dispersion intervals were measured from standard 12-lead electrocardiography. No statistically significant differences were found between the groups in QT dispersion, corrected QT dispersion, and systolic-diastolic function of the left ventricle parameters. During the 12 months of follow-up, no ventricular arrhythmias were documented in either group.

Echocardiographic Follow-Up of Children with Supravalvular Aortic Stenosis

This study evaluates the course of supravalvular aortic stenosis (SVAS)-associated right ventricular outflow tract (RVOT) obstruction and the results of surgery in children. We reviewed the medical records of 24 patients diagnosed with SVAS at initial echocardiographic examination or during the following period of RVOT obstruction. Very mild SVAS was defined as a transvalvular Doppler peak systolic instantanous gradient (PSIG) less than 25 mmHg, mild stenosis as 25–49 mmHg, moderate stenosis as 50–75 mmHg, and severe stenosis as more than 75 mmHg. The mean age of the patients was 3.1 ± 2.9 years (range, 7 days to 12.7 years), and 18 of the patients (72%) were male. Fifteen patients had Williams’ syndrome. Seventeen patients (71%) were followed for a mean of 5.2 ± 3.8 years (range, 7 months to 13.5 years). Among 17 patients with complete follow-up records, 1 (6%) had very mild, 5 (29%) mild, 3 (18%) moderate, and 3 (18%) severe aortic stenosis at initial echocardiographic examination. In a newborn patient with mild pulmonary valvular stenosis. SVAS became evident after 2 months and progressed rapidly. Supravalvular aortic stenosis was very mild in 4 patients (23%), mild in 3 (18%), moderate in 3 (18%), and severe in 7 (41%) at last echocardiographic examination. Of 17 patients who were followed, 11 (65%) had RVOT obstruction at initial echocardiographic examination. RVOT obstruction disappeared in 5 patients, regressed in 1 patient, and appeared in 1 patient over the follow-up period. Four patients underwent operation. It appears reasonable that patients with very mild and mild stenosis should be followed medically every 1 or 2 years and patients with moderate stenosis once a year. Newborns with SVAS should be followed for rapid progression of SVAS. In some patients, RVOT obstruction may disappear, and SVAS may develop in others with RVOT obstruction. Patients with RVOT obstruction (at the valvular, supravalvular, or peripheral pulmonary arterial level) should be evaluated carefully for development of SVAS at follow-up.

Evaluation of right ventricular function in patients with tetralogy of Fallot using the myocardial performance index and isovolumic acceleration: a comparison with cardiac magnetic resonance imaging.

BACKGROUND Assessment of right ventricular function is a key point in the follow-up of operated patients with tetralogy of Fallot. Cardiac magnetic resonance assessment of right ventricular function is considered the gold standard. However, this technique is expensive, has limited availability, and requires significant expertise to acquire and interpret the images. Myocardial performance index and isovolumic acceleration have recently been studied for the assessment of right ventricular function and are shown to be simple yet powerful tools for assessing patients with right ventricular dysfunction of various origins. METHODS In this study, the integrity of myocardial performance index and isovolumic acceleration obtained by tissue Doppler imaging echocardiography to quantify right ventricular function was assessed in 31 patients operated for tetralogy of Fallot. Myocardial performance index and isovolumic acceleration measurements were compared with the parameters derived by cardiac magnetic resonance imaging. RESULTS In this study, a significant correlation has not been detected between cardiac magnetic resonance-originated right ventricular ejection fraction, pulmonary regurgitation fraction and myocardial performance index, isovolumic acceleration obtained by tissue Doppler imaging echocardiography from the lateral tricuspid annulus of the right ventricle. CONCLUSION We have concluded that when evaluated separately, myocardial performance index and isovolumic acceleration obtained from tissue Doppler imaging echocardiography can be used in the long-term follow-up of patients who have been operated for tetralogy of Fallot, but that they do not show correlation with cardiac magnetic resonance-originated right ventricle ejection fraction and pulmonary regurgitation fraction.