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.

Predictive value of cardiothoracic ratio as a marker of severity of aortic regurgitation and mitral regurgitation.

OBJECTIVE: In this study we compared cardiothoracic ratio on chest radiography and left ventricular dimensions from echocardiography in patients with left heart valvular regurgitation. METHODS: The studied population consisted of 107 patients (55 male, 52 female) aged 7 to 25 years (11.6+/-4.7 years) with isolated mitral or aortic regurgitation. Chest radiography and echocardiographic examination were performed on the same day in every patient. RESULTS: Among 26 patients with moderate mitral regurgitation, cardiac enlargement was found in 4 (15%) patients on chest radiography, and in 7 (27%) patients on echocardiography. Among 25 patients with severe mitral regurgitation, cardiothoracic ratio was normal in 20 (80%) patients whereas cardiac enlargement was documented in 17 (68%) patients on echocardiography. Although there was no patient with cardiac enlargement (CE) on chest radiography in the groups of mild and moderate aortic regurgitation, 50% of patients in the group of severe aortic regurgitation had CE on chest radiography; cardiac enlargement was detected in 62% patients with moderate and 100% patients with severe aortic regurgitation on echocardiography. We found a good relation between the severity of valvular regurgitation, especially for aortic regurgitation, and CE on echocardiography; however only a poor relation was detected between the severity of valvular regurgitation and CE on chest radiography. CONCLUSION: In conclusion, prediction of severity of valvular regurgitation using chest radiography may lead to false interpretations and so, plain chest radiography may not be an essential part of the routine evaluation of such patients.

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.

A different type of cardiomyopathy: ventricular noncompaction (evaluation of 8 cases).

Ventricular noncompaction, characterized by numerous, prominent ventricular trabeculations and deep intratrabecular recesses, is thought to be due to an arrest of myocardial morphogenesis. We report eight patients with ventricular noncompaction diagnosed at our center in the previous one year. Two patients had associated congenital cardiac anomalies while the others were without coexisting cardiac abnormalities. Both ventricles were involved in one patient, only the right ventricle in one, and only the left ventricle in six patients. Seven patients had initially been diagnosed as having different types of cardiomyopathies. In conclusion, based on our limited experience, we propose that during the differential diagnosis of cardiomyopathies, ventricular noncompaction should be considered.

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.

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.

Venous and intrapericardial thrombosis: secondary to transient protein C deficiency.

We present a 2-month-old male infant with thrombosis in the superior vena cava and pericardium due to transient protein C deficiency. Protein C deficiency was related to sepsis and hepatitis-induced liver function impairment. The patient's cardiac anatomy was otherwise normal. The patient was referred to us with signs of superior vena cava syndrome. Pericardial mass was excised. Pathological examination diagnosed the mass as organized thrombus. After the operation, signs of superior vena cava syndrome totally resolved. Serial echocardiographic examinations revealed regression of thrombus in the superior vena cava. This is the first case reported in the literature with intrapericardial thrombus secondary to transient protein C deficiency.

[Fetal arrhythmias: diagnosis, treatment and prognosis]. / Fetal Aritmiler: Tani, Tedavi, Prognoz.

OBJECTIVE: To evaluate patients who were referred to our center with suspicion of fetal arrhythmia for diagnosis, results of treatment and prognosis. METHODS: Thirty-three patients referred to our center for evaluation of fetal cardiac arrhythmias were examined by M-mode, two-dimensional and Doppler echocardiography for cardiac anomaly and type of dysrhythmia RESULTS: Arrhythmias were diagnosed in 15 of 33 patients. Four cases demonstrated tachycardia, 5--ectopic beats, 6--bradycardia. Of 5 fetuses with tachycardia, transplacental antiarrhythmic drugs were administered in three cases; two of them with hydrops died and conversion of the arrhythmia was achieved in one. Six patients with bradycardia had poor perinatal outcome, with 3 deaths occurred (two of them had third degree atrioventricular block, one of them had sinus bradycardia). All patients with ectopic beats had a good perinatal outcome. CONCLUSION: The results suggest that bradycardic and/or hydropic fetuses have poor prognosis, however fetuses with ectopic beats generally have desirable prognosis.