Gülay Ahunbay

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).

The Natural Course of Isolated Ventricular Septal Defect During Adolescence

Abstract. Serial changes in patients with isolated VSD during adolescence have not previously been investigated. Hemodynamic status, diameter of the defect, and growth were studied yearly in 106 children with VSD. The mean duration of the follow-up was 13.16 years and ranged in 80% of subjects from 7 to 19 years (1395 patient years). The mean ages at pre- and postpuberty were 8.62 and 16.67, respectively. The presented longitudinal study, in which losses due to death and operation were minimal (4%), ideally reflected the natural history of VSD. Although weight showed retardation during prepuberty, this lag was caught up by the end of adolescence. Stature showed no retardation in pre- and postpuberty. Cardiothoracic ratio decreased significantly from a mean of 0.48 to 0.44 and showed normal variation. Although the mean defect diameter at prepuberty was 5.33 mm, this decreased to 2.7 postpubertally. The individual decrease (1.7 ± 2.34 mm) was significant (t= 5.349, p < 1/105). The defect closed spontaneously in 24 (22.6%). In the 75 patients without pulmonary hypertension and with mild left-to-right shunting, 52 remained in the same class and spontaneous closure was observed in 23. In the 24 patients with moderate to severe left-to-right shunt, this decreased in 23 and only one remained stable. The 2 patients (1.9%) with Eisenmenger syndrome remained stable and 1 died. Aortic regurgitution developed in 10 patients (9.4%); however, this was of mild degree in most of them. No infective endocarditis was observed. It is concluded that patients with VSD should be followed closely through adolescence, because the diameter of the defect, as well as left-to-right shunting, can decrease, and it is concluded that the spontaneous closure of the defect is to be expected in a considerable 23%, and aortic prolapse or mild regurgitation may develop in approximately 10%.

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.

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.

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.

Regression of right ventricular pressure in ventricular septal defect in infancy: a longitudinal color-flow Doppler echocardiographic study.

Abstract. To determine the course of right ventricular pressure (RVP) in patients with isolated ventricular septal defect (VSD) and factors influencing it, unselected 148 infants were followed-up longitudinally with color-Doppler echocardiography from a median age of 1 month for 201 patient-years. The patients were divided into three groups by absolute echographic size of VSD: group I, ≤4.0 mm; group II, >4 to ≤7 mm; group III, >7 mm. Sixty percent belonged to group I. Muscular defects dominated in group I, perimembranous defects dominated in group II, and those with outlet extensions dominated in group III. Peak systolic RVP was obtained by Doppler-estimated difference between systolic brachial artery and peak gradient across the VSD. Initial RVP ranged between 15 and 95 mmHg and increased in parallel to the size of defect. According to the regression equations RVP decreased in general by 0.17 mmHg per month. This correlated significantly with the size of the defect. In group I, the rate of decrease was very fast and is best expressed by a log function of time (r=−0.67, r2= 0.45). In groups II and III the rate of decrease was less steep and had a greater variability. RVP normalized in 100% in those of group I and in 90% of group II, at median ages of 0.17 and 0.33 years, respectively. Median Qp:Qs values were 1.5, 2.2, and 3.0 in groups I–III, respectively. The outcome depended on the size of VSD. Spontaneous closure was observed in 51% of group I, 10% of group II, and none of group III. The rate was higher in muscular defects. Congestive heart failure was present in 53% and 100% in groups II and III, respectively. Death rate was 2.03%, all in patients with large defects. It is concluded that the temporal course of RVP with time can be estimated fairly well by the regression equation presented in relation to the initial size of the VSD.

Regression of right ventricular dominance in the electrocardiogram after birth: a longitudinal follow-up of a healthy cohort from birth to 3 years.

Abstract. To establish the incidence and course of right ventricular (RV) conduction delay during the regression of right ventricular dominance, 12 serial ECGs were recorded at regular intervals until age 36 months in a cohort of 43 normal neonates. Additionally, ECGs were recorded similarly in 6 infants from the age of 1 month and in 7 from 2 months. The right precordial R waves were classified according to their amplitudes and their occurrence before or after the S wave. QR duration and its ratio to QRS were measured in right and left chest leads. The incidence of notched R waves, which is very rare in neonates, increased after the first month and occurred before the peak of R. Thereafter, the height of the notch decreased and then occurred after the peak of R. This turned into an r′ (after the S wave) and finally the amplitude of r′ decreased further and evolved into a notched S pattern. An rSR′ or qRS pattern in the right precordial leads was never observed. An abnormal incomplete right bundle branch block during infancy can be distinguished from physiological right ventricular conduction delay by the unvarying amplitude of the R′ wave, preceded by an S wave.