To describe the prenatal diagnosis and outcome of fetal cardiomyopathy (CM). The charts, photogra... more To describe the prenatal diagnosis and outcome of fetal cardiomyopathy (CM). The charts, photographs and videotapes of all fetuses with CM, who were assessed during pregnancy at two referral centers, were reviewed. The diagnosis of CM was established in 12 fetuses. All had structurally normal hearts, and all cases were diagnosed after 23 weeks of gestation, following normal early fetal echocardiogram. Three clusters of fetal CM appeared: Familial--two sib fetuses of a mother, who is a second generation of CM. Both had dilated CM and pathological findings were consistent with the diagnosis of endocardial fibroelastosis. Secondary--CM that was induced by another factor. Idiopathic--six cases of CM without an underlying specific etiology. Three women elected to terminate their pregnancy. Among the nine who delivered, four had a favorable outcome with normal cardiac function at the age of 1 month, in which three belonged to the secondary category, and five cases were complicated by fetal/infant death. CM may develop during fetal life and might be diagnosed by prenatal echocardiography. Normal cardiac findings in a midtrimester fetus do not exclude subsequent development of CM. Detailed prenatal sonographic examination may aid in determining the neonatal outcome.
Scimitar syndrome is a rare congenital anomaly that consists of secondary dextroposition in situs... more Scimitar syndrome is a rare congenital anomaly that consists of secondary dextroposition in situs solitus, due to right pulmonary hypoplasia. The prenatal sonographic findings of this abnormality have not been previously described. We describe early sonographic findings in a fetus that included isolated cardiac dextroposition with normal abdominal situs, hydramnios and mild narrowing of the right pulmonary artery. Postnatally, the infant developed heart failure, and a right hypoplastic lung was found. Catheterization revealed collateral supply to the right lung arising from the descending aorta and anomalous drainage of right pulmonary veins to the inferior vena cava. The diagnosis of scimitar syndrome was therefore established. A coil embolization of arterial collaterals to the right lung was performed. Scimitar syndrome should be considered in a fetus with the sonographic findings of a right shift of the mediastinal structures, an intact diaphragm and narrow right pulmonary artery. This may allow early neonatal stabilization and treatment.
To evaluate the prenatal parameters that increase the risk of tuberous sclerosis in prenatal mana... more To evaluate the prenatal parameters that increase the risk of tuberous sclerosis in prenatal management of fetal cardiac tumors suspected as rhabdomyoma. The study was a retrospective survey of 18 documented cases in which cardiac rhabdomyoma was suspected during pregnancy. The following parameters were evaluated as possible risk factors associated with tuberous sclerosis: tumor size, isolated or multiple, and family history of tuberous sclerosis. Eighteen documented cases in which cardiac rhabdomyoma was found during pregnancy were evaluated. Of these cases, seven (39%) had proven tuberous sclerosis and 11 were found to be non-associated tuberous sclerosis tumors. When combining the present data with previous series, cases with diagnosis of tuberous sclerosis had equal mean tumor size to those with normal outcome. Family history of tuberous sclerosis in the presence of cardiac rhabdomyoma almost invariably ended with tuberous sclerosis (86%). All other cases with diagnosis of tuberous sclerosis and no family history had all multiple cardiac tumors. The present data suggest that 39% of in utero suspected cardiac rhabdomyoma would have tuberous sclerosis. Family history and multifocality remain the strongest predictors of tuberous sclerosis, whereas size of the cardiac tumor can not reliably be used to predict tuberous sclerosis in prenatal counseling.
Vein of Galen aneurysmal malformations are rarely seen intracranial malformations. They represent... more Vein of Galen aneurysmal malformations are rarely seen intracranial malformations. They represent less than 1% of the cerebral arteriovenous malformations. Prenatal diagnosis of an arteriovenous fistula malformation may be achieved by real-time and Doppler sonography with color flow imaging, by identifying dilated veins and arteriovenous shunts with turbulent flow. In addition, an elevated cardiac output may be observed and correlated with the magnitude of the cerebral arteriovenous shunt. The high incidence of cardiomegaly in neonates with arteriovenous malformations also suggests that high-output cardiac failure is already present in a significant number of cases during the 3rd trimester, and, therefore, treatment in utero may need to be considered to improve the hemodynamic status. In our case, all the above cardiovascular features were present during prenatal ultrasonography. The information on outcome compiled from the literature suggests that when an arteriovenous malformation...
Objective: To describe our experience with late first and early second trimester, prenatal diagno... more Objective: To describe our experience with late first and early second trimester, prenatal diagnosis of abnormalities in the three-vessels and tracheal view (3VT) of the heart, utilizing high-resolution ultrasonography and color Doppler techniques. Materials and Methods: Fetuses with structural heart defects detected at late first and early second-trimester prenatal screening, which were diagnosed with an abnormality on the 3VT. Color Doppler was added in all cases. All patients received genetic counselling and pediatric cardiology consultation. Results: Twenty-one fetuses had an abnormality detected on the three-vessels and tracheal view. The median gestational age was 15 weeks (range 12-18). Lesions diagnosed included abnormal aortic size (hypoplastic left heart syndrome, narrow aorta, coarctation of the aorta); right aortic arch; abnormal pulmonary artery size (tetralogy of Fallot, pulmonary atresia); abnormal vessels arrangement (transposition of the great arteries); two vessels...
The results of membranectomy and deep myectomy in the left ventricular outflow tract were compare... more The results of membranectomy and deep myectomy in the left ventricular outflow tract were compared to those of membranectomy and myotomy in 42 patients who underwent surgical repair of discrete and tunnel subaortic stenosis. Fifteen consecutive patients (Group A) underwent membranectomy and myotomy, and 27 consecutive patients (Group B) underwent membranectomy and myectomy. Two patients of Group A and nine of Group B had tunnel subaortic stenosis. The preoperative mean (+/- standard deviation) peak systolic gradients across the left ventricular outflow tract in patients with discrete subaortic stenosis types I and II were 64 +/- 29 mm Hg in Group A and 52 +/- 3 mm Hg in Group B (p = not significant). In the patients with tunnel subaortic stenosis the preoperative mean gradients were 97 +/- 74 mm Hg in Group A and 73 +/- 26 mm Hg in Group B (p = not significant). In patients with discrete subaortic stenosis types I and II, postoperative catheterization at a mean follow-up of 21 months revealed residual mean gradients of 29 +/- 24 mm Hg in Group A and 10 +/- 13 mm Hg in Group B (p less than 0.01). In the patients with tunnel subaortic stenosis, the postoperative mean gradients were 25 +/- 7 and 30 +/- 30 mm Hg in Groups A and B, respectively (p = not significant). We conclude that in the surgical management of discrete subaortic stenosis types I and II, deep myectomy (in addition to membranectomy) produces better relief of the left ventricular outflow obstruction than do membranectomy and myotomy. In patients with tunnel subaortic stenosis myectomy is less effective than in the non-tunnel type but still produces acceptable results and may delay radical procedures to a later age.
Congestive heart failure has never been described in patients with dysplastic stenotic pulmonary ... more Congestive heart failure has never been described in patients with dysplastic stenotic pulmonary valve without associated shunt lesions. We describe two patients with mild pulmonic stenosis due to valvular dysplasia associated with cardiomyopathy who developed severe congestive heart failure. Since the small pressure gradients across the pulmonary valve cannot cause this complication, we suggest that it resulted from the associated hypertrophic non-obstructive cardiomyopathy. The presence of cardiomyopathy may alter the clinical presentation and prognosis of patients with dysplastic pulmonary valve. In some cases, like our two cases, the cardiomyopathy rather than the valvular lesion should be considered the main disease.
Over the 3 year period from 1980 to 1982, mechanical prosthetic valves were implanted in the pulm... more Over the 3 year period from 1980 to 1982, mechanical prosthetic valves were implanted in the pulmonary pathway in seven patients. In two the procedure was a pulmonary valve replacement. In two it was an implantation of a pulmonary valve with transannular pericardial patching, and in three, complete pericardial (tube) conduits, which included mechanical valves, were constructed. There were neither perioperative nor late complications. Four patients were recatheterized. In three residual right ventricular hypertension was observed. In one of the three the cause of obstruction was not verified, but in the remaining two it was unrelated to the mechanical valve. No thromboembolic phenomena were observed.
A term fetus with atrial flutter and no signs of cardiac decompensation is described. A trial of ... more A term fetus with atrial flutter and no signs of cardiac decompensation is described. A trial of pharmacologic cardioversion was unsuccessful, and induction of labor was initiated. Immediately after artificial rupture of the membranes, spontaneous conversion to normal sinus rhythm occurred. The possible mechanism of the cardioverion and the optional treatments for the term fetus with atrial flutter are discussed.
To describe the prenatal diagnosis and outcome of fetal cardiomyopathy (CM). The charts, photogra... more To describe the prenatal diagnosis and outcome of fetal cardiomyopathy (CM). The charts, photographs and videotapes of all fetuses with CM, who were assessed during pregnancy at two referral centers, were reviewed. The diagnosis of CM was established in 12 fetuses. All had structurally normal hearts, and all cases were diagnosed after 23 weeks of gestation, following normal early fetal echocardiogram. Three clusters of fetal CM appeared: Familial--two sib fetuses of a mother, who is a second generation of CM. Both had dilated CM and pathological findings were consistent with the diagnosis of endocardial fibroelastosis. Secondary--CM that was induced by another factor. Idiopathic--six cases of CM without an underlying specific etiology. Three women elected to terminate their pregnancy. Among the nine who delivered, four had a favorable outcome with normal cardiac function at the age of 1 month, in which three belonged to the secondary category, and five cases were complicated by fetal/infant death. CM may develop during fetal life and might be diagnosed by prenatal echocardiography. Normal cardiac findings in a midtrimester fetus do not exclude subsequent development of CM. Detailed prenatal sonographic examination may aid in determining the neonatal outcome.
Scimitar syndrome is a rare congenital anomaly that consists of secondary dextroposition in situs... more Scimitar syndrome is a rare congenital anomaly that consists of secondary dextroposition in situs solitus, due to right pulmonary hypoplasia. The prenatal sonographic findings of this abnormality have not been previously described. We describe early sonographic findings in a fetus that included isolated cardiac dextroposition with normal abdominal situs, hydramnios and mild narrowing of the right pulmonary artery. Postnatally, the infant developed heart failure, and a right hypoplastic lung was found. Catheterization revealed collateral supply to the right lung arising from the descending aorta and anomalous drainage of right pulmonary veins to the inferior vena cava. The diagnosis of scimitar syndrome was therefore established. A coil embolization of arterial collaterals to the right lung was performed. Scimitar syndrome should be considered in a fetus with the sonographic findings of a right shift of the mediastinal structures, an intact diaphragm and narrow right pulmonary artery. This may allow early neonatal stabilization and treatment.
To evaluate the prenatal parameters that increase the risk of tuberous sclerosis in prenatal mana... more To evaluate the prenatal parameters that increase the risk of tuberous sclerosis in prenatal management of fetal cardiac tumors suspected as rhabdomyoma. The study was a retrospective survey of 18 documented cases in which cardiac rhabdomyoma was suspected during pregnancy. The following parameters were evaluated as possible risk factors associated with tuberous sclerosis: tumor size, isolated or multiple, and family history of tuberous sclerosis. Eighteen documented cases in which cardiac rhabdomyoma was found during pregnancy were evaluated. Of these cases, seven (39%) had proven tuberous sclerosis and 11 were found to be non-associated tuberous sclerosis tumors. When combining the present data with previous series, cases with diagnosis of tuberous sclerosis had equal mean tumor size to those with normal outcome. Family history of tuberous sclerosis in the presence of cardiac rhabdomyoma almost invariably ended with tuberous sclerosis (86%). All other cases with diagnosis of tuberous sclerosis and no family history had all multiple cardiac tumors. The present data suggest that 39% of in utero suspected cardiac rhabdomyoma would have tuberous sclerosis. Family history and multifocality remain the strongest predictors of tuberous sclerosis, whereas size of the cardiac tumor can not reliably be used to predict tuberous sclerosis in prenatal counseling.
Vein of Galen aneurysmal malformations are rarely seen intracranial malformations. They represent... more Vein of Galen aneurysmal malformations are rarely seen intracranial malformations. They represent less than 1% of the cerebral arteriovenous malformations. Prenatal diagnosis of an arteriovenous fistula malformation may be achieved by real-time and Doppler sonography with color flow imaging, by identifying dilated veins and arteriovenous shunts with turbulent flow. In addition, an elevated cardiac output may be observed and correlated with the magnitude of the cerebral arteriovenous shunt. The high incidence of cardiomegaly in neonates with arteriovenous malformations also suggests that high-output cardiac failure is already present in a significant number of cases during the 3rd trimester, and, therefore, treatment in utero may need to be considered to improve the hemodynamic status. In our case, all the above cardiovascular features were present during prenatal ultrasonography. The information on outcome compiled from the literature suggests that when an arteriovenous malformation...
Objective: To describe our experience with late first and early second trimester, prenatal diagno... more Objective: To describe our experience with late first and early second trimester, prenatal diagnosis of abnormalities in the three-vessels and tracheal view (3VT) of the heart, utilizing high-resolution ultrasonography and color Doppler techniques. Materials and Methods: Fetuses with structural heart defects detected at late first and early second-trimester prenatal screening, which were diagnosed with an abnormality on the 3VT. Color Doppler was added in all cases. All patients received genetic counselling and pediatric cardiology consultation. Results: Twenty-one fetuses had an abnormality detected on the three-vessels and tracheal view. The median gestational age was 15 weeks (range 12-18). Lesions diagnosed included abnormal aortic size (hypoplastic left heart syndrome, narrow aorta, coarctation of the aorta); right aortic arch; abnormal pulmonary artery size (tetralogy of Fallot, pulmonary atresia); abnormal vessels arrangement (transposition of the great arteries); two vessels...
The results of membranectomy and deep myectomy in the left ventricular outflow tract were compare... more The results of membranectomy and deep myectomy in the left ventricular outflow tract were compared to those of membranectomy and myotomy in 42 patients who underwent surgical repair of discrete and tunnel subaortic stenosis. Fifteen consecutive patients (Group A) underwent membranectomy and myotomy, and 27 consecutive patients (Group B) underwent membranectomy and myectomy. Two patients of Group A and nine of Group B had tunnel subaortic stenosis. The preoperative mean (+/- standard deviation) peak systolic gradients across the left ventricular outflow tract in patients with discrete subaortic stenosis types I and II were 64 +/- 29 mm Hg in Group A and 52 +/- 3 mm Hg in Group B (p = not significant). In the patients with tunnel subaortic stenosis the preoperative mean gradients were 97 +/- 74 mm Hg in Group A and 73 +/- 26 mm Hg in Group B (p = not significant). In patients with discrete subaortic stenosis types I and II, postoperative catheterization at a mean follow-up of 21 months revealed residual mean gradients of 29 +/- 24 mm Hg in Group A and 10 +/- 13 mm Hg in Group B (p less than 0.01). In the patients with tunnel subaortic stenosis, the postoperative mean gradients were 25 +/- 7 and 30 +/- 30 mm Hg in Groups A and B, respectively (p = not significant). We conclude that in the surgical management of discrete subaortic stenosis types I and II, deep myectomy (in addition to membranectomy) produces better relief of the left ventricular outflow obstruction than do membranectomy and myotomy. In patients with tunnel subaortic stenosis myectomy is less effective than in the non-tunnel type but still produces acceptable results and may delay radical procedures to a later age.
Congestive heart failure has never been described in patients with dysplastic stenotic pulmonary ... more Congestive heart failure has never been described in patients with dysplastic stenotic pulmonary valve without associated shunt lesions. We describe two patients with mild pulmonic stenosis due to valvular dysplasia associated with cardiomyopathy who developed severe congestive heart failure. Since the small pressure gradients across the pulmonary valve cannot cause this complication, we suggest that it resulted from the associated hypertrophic non-obstructive cardiomyopathy. The presence of cardiomyopathy may alter the clinical presentation and prognosis of patients with dysplastic pulmonary valve. In some cases, like our two cases, the cardiomyopathy rather than the valvular lesion should be considered the main disease.
Over the 3 year period from 1980 to 1982, mechanical prosthetic valves were implanted in the pulm... more Over the 3 year period from 1980 to 1982, mechanical prosthetic valves were implanted in the pulmonary pathway in seven patients. In two the procedure was a pulmonary valve replacement. In two it was an implantation of a pulmonary valve with transannular pericardial patching, and in three, complete pericardial (tube) conduits, which included mechanical valves, were constructed. There were neither perioperative nor late complications. Four patients were recatheterized. In three residual right ventricular hypertension was observed. In one of the three the cause of obstruction was not verified, but in the remaining two it was unrelated to the mechanical valve. No thromboembolic phenomena were observed.
A term fetus with atrial flutter and no signs of cardiac decompensation is described. A trial of ... more A term fetus with atrial flutter and no signs of cardiac decompensation is described. A trial of pharmacologic cardioversion was unsuccessful, and induction of labor was initiated. Immediately after artificial rupture of the membranes, spontaneous conversion to normal sinus rhythm occurred. The possible mechanism of the cardioverion and the optional treatments for the term fetus with atrial flutter are discussed.
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