P32.04: Left ventricular aneurysm as an antenatal ultrasonographic marker of hypertrophic cardiomyopathy: a case report

17th World Congress on Ultrasound in Obstetrics and Gynecology

Poster abstracts

RAA with mirror-image branching. Only one case had deletion of chromosome 22q11. In this case, omphalocele was also found. In two of the cases of RAA with mirror-image branching, the parents chose to terminate the pregnancy. One case of RAA with mirrorimage branching was born alive and received corrective surgery for TOF. In the case of RAA with an aberrant left subclavian artery, no other associated anomalies were recognized despite the vascular ring being formed in the prenatal period. This case was born alive and did well with spontaneous closure of the ductus arteriosus. Conclusion: Prenatal detection of RAA is feasible by 3 VT view. RAA combined with TOF is very common in RAA with mirror-image branching. Meticulous cardiac and extra-cardiac examination with invasive testing seems to be mandatory in prenatal diagnosis of RAA.

Methods: We reviewed fetal echocardiography from July 1996 to March 2007 at our center. We had 1697 congenital heart anomalies and suspected TAPVC in 47. We compared prenatal with postnatal echocardiography or autopsy findings. Results: Among 47 fetuses, we could identify TAPVC in 37, 24 through postnatal echocardiography and 13 in the autopsy record, eight have not shown up again and two have not delivered yet. We suspected TAPVC if pulmonary vein to left atrium was not definite or an extrachamber was shown in 2D and color Doppler ultrasound. Out of 24 deliveries, nine were confirmed as TAPVC, one PAPVC, and one pulmonary venous stenosis. Through autopsy, two out of 13 were confirmed as TAPVC and one PAPVC. Total 11 TAPVC showed six supracardiac, one cardiac, three infracardiac, and one mixed connection. Three deaths occurred due to postoperative acidosis, septic shock, and congestive heart failure. There was no chromosomal anomaly. We suspected isolated TAPVC in four prenatally, which were proven as three TAPVC and one pulmonary venous stenosis. The distinguishable point was that we could not see any extrachamber in the latter. The isolated TAPVCs are in a good state after surgery, but the fetus with stenotic pulmonary vein expired one day after birth. Conclusions: In 2D or color Doppler ultrasound prenatally, absence of typical pulmonary drain to left atrium or presence of an extrachamber could provide simple clues for anomalous pulmonary venous connection. Indefinite extrachamber could not rule out anomalous pulmonary venous connection. On the contrary, in that case, pulmonary venous stenosis should be suspected, which seemed most important in prognosis.

P32.04 Left ventricular aneurysm as an antenatal ultrasonographic marker of hypertrophic cardiomyopathy: a case report R. A. Marquez, H. Schilling, A. Scarella, M. Rodriguez, G. Egana, D. Badilla Universidad de Valparaiso, Chile Hypertrophic cardiomyopathy (HCM) is a genetic disease of the cardiac sarcomere. The hallmark of the disorder is myocardial hypertrophy that is inappropriate and occurs in the absence of an obvious hypertrophy stimulus. The disorder has a variable presentation and is the leading cause of sudden death in preadolescents. We report a case of a left ventricular aneurysm, antenatally diagnosed that was confirmed, postnatally, as a HCM. To the best of our knowledge, a ventricular aneurysm has not been reported in association with HCM in a newborn before. A 18-year-old gravida 1, para 0, presented late for prenatal care at 31 weeks’ gestation with no previous pregnancy complications or history of diabetes. A fetal echocardiogram examination at 31 weeks showed a 28 × 24 mm ventricular aneurysm located in the inferior left heart chamber. Doppler, M mode and the remainder of the cardiac and extra cardiac anatomy was normal. There were no signs of fetal hydrops, ventricular function deterioration or increase in aneurysm size in later echocardiograms performed. Elective Cesarean section was performed at 40 weeks’ gestation. A female infant weighing 3360 g was born. Apgar scores of 9 and 9 at 1 and 5 min. The adaptation to extrauterine life was normal. We could hear a systolic murmur and the electrocardiogram of the infant informed a left ventricular hypertrophy. The echocardiogram showed an enlarged left ventricle with a large aneurysm and hypokinesia. Myocardial enzymes were normal. After 11 months the patient is in good clinical condition, asymptomatic, growth is normal and surgery has not been performed. Congenital left ventricular aneurysm is an outpouching of the ventricular wall. The etiopathogenesis is unknown and it has been reported, as an antenatal diagnosis, only ten times so far. Between the hypotheses proposed we find myocardial ischemia and developmental myocardial anomaly. We report one case of a left ventricular aneurysm that behaves as an antenatal marker of HCM.

P32.05 Prenatal echocardiography of total anomalous pulmonary venous connection (TAPVC) : isolated or accompanied by pulmonary venous stenosis E. S. Choi, H. S. Won, J. Y. Shim, P. R. Lee, A. Kim

P32.06 Multiple cardiac rhabdomyomas R. Radulescu-Botica, D. Pelinescu-Onciul, A. Simionescu, L. Giurcaneanu Filantropia Clinical Hospital, Romania We present the case of a 30-year-old woman gravida 1, para 1, admitted at 30 weeks of gestation for ultrasound examination. At ultrasound scan we found that all biophysical parameters were in normal range for this gestational age. Morphological appearance was normal except for two fetal intracardiac tumors located in the left ventricle and a muscular ventricular septal defect of 3.2 mm. A 20 week scan was completely normal. Intracardiac tumors originated from the ventricular wall and had a homogeneous hyperechogenic appearance without visible vascularisation. These ultrasonographic findings are consistent with rhabdomyomas. These intracardiac tumors accounts for up to 90% of prenatally diagnosed cardiac tumors and one half are multiple. 50% of fetuses with rhabdomyomas have also associated tuberous sclerosis, an autosomal dominant disorder linked with chromosome 9. We did not find any hydrops or cardiac arrhythmias as well as renal or cerebral anomalies that could be associated with tuberous sclerosis. The child was born by Cesarean section and had a normal morphological appearance and hemodynamic parameters except for a holosystolic murmur. Most rhabdomyomas regress postnatally and in the case that no complications occur in the first 6 months of life there is a low risk of creating problems later. This case report emphasizes the importance of the ultrasound examination around the beginning of the third trimester because some conditions that develop around the end of second trimester and the beginning of third trimester of pregnancy could therefore be recognized early, monitored and could receive the best management until and after delivery.

Asan Medical Center, Republic of Korea Objectives: TAPVC deteriorates the neonatal condition. It is usually accompanied by other cardiac anomalies. While isolated TAPVC could have an excellent outcome if detected and corrected early in life, TAPVC with pulmonary venous stenosis would have a much worse result. It is important to detect TAPVC prenatally and the associated pulmonary venous stenosis as well.

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P32.07 Fetal cardiac rhabdomyoma diagnosed at twenty weeks of gestation: a case report ´ R. Ros M. Rabanal, R. Campos, A. Filloy, M. Bastus, Hospital Sant Joan de Deu de Martorell, Spain

Ultrasound in Obstetrics & Gynecology 2007; 30: 547–653