scholarly journals Prenatal diagnosis, associated findings and postnatal outcome of fetuses with truncus arteriosus communis (TAC)

2021 ◽  
Author(s):  
J. S. Abel ◽  
C. Berg ◽  
A. Geipel ◽  
U. Gembruch ◽  
U. Herberg ◽  
...  
Keyword(s):  
Prenatal Diagnosis ◽  
Health Status ◽  
Pulmonary Artery ◽  
Truncus Arteriosus ◽  
Intrauterine Fetal Death ◽  
Cardiac Anomaly ◽  
Chromosomal Anomalies ◽  
Cardiac Anomalies ◽  
Postnatal Outcome

Abstract Purpose To assess the spectrum of associated anomalies, the intrauterine course, postnatal outcome and management of fetuses with truncus arteriosus communis (TAC) Methods All cases of TAC diagnosed prenatally over a period of 8 years were retrospectively collected in two tertiary referral centers. All additional prenatal findings were assessed and correlated with the outcome. The accuracy of prenatal diagnosis was assessed. Results 39 cases of TAC were diagnosed prenatally. Mean gestational age at first diagnosis was 22 weeks (range, 13–38). Two cases were lost follow-up. Correct prenatal diagnosis of TAC was made in 21 of 24 (87.5%) cases and of TAC subtype in 19 of 21 (90.5%) cases. Prenatal diagnosis of TAC was incorrect in three cases: one newborn had aortic atresia with ventricular septal defect postnatally, one had hypoplastic right ventricle with dextro Transposition of the Great Arteries with coartation of the aorta and a third newborn had Tetralogy of Fallot with abnormal origin of the left pulmonary artery arising from the ascending aorta postnatally. These three cases were excluded from further analysis. In 9 of 34 (26.5%) cases, TAC was an isolated finding. 13 (38.2%) fetuses had additional chromosomal anomalies. Among them, microdeletion 22q11.2 was most common with a prevalence of 17.6% in our cohort. Another 3 fetuses were highly suspicious for non-chromosomal genetic syndromes due to their additional extra-cardiac anomalies, but molecular diagnosis could not be provided. Major cardiac and extra-cardiac anomalies occurred in 3 (8.8%) and in 20 (58.8%) cases, respectively. Predominantly, extra-cardiac anomalies occurred in association with chromosomal anomalies. Additionally, severe IUGR occurred in 6 (17.6%) cases. There were 14 terminations of pregnancy (41.2%), 1 (2.9%) intrauterine fetal death, 5 postnatal deaths (14.7%) and 14 (41.2%) infants were alive at last follow-up. Intention-to-treat survival rate was 70%. Mean follow-up among survivors was 42 months (range, 6–104). Postoperative health status among survivors was excellent in 11 (78.6%) infants, but 5 (46.2%) of them needed repeated re-interventions due to recurrent pulmonary artery or conduit stenosis. The other 3 (21.4%) survivors were significantly impaired due to non-cardiac problems. Conclusion TAC is a rare and complex cardiac anomaly that can be diagnosed prenatally with high precision. TAC is frequently associated with chromosomal and extra-cardiac anomalies, leading to a high intrauterine and postnatal loss rate due to terminations and perioperative mortality. Without severe extra-cardiac anomalies, postoperative short- and medium-term health status is excellent, independent of the subtype of TAC, but the prevalence of repeated interventions due to recurrent stenosis is high.

scholarly journals Prenatal diagnosis, associated findings and postnatal outcome of fetuses with truncus arteriosus communis (TAC)

2021 ◽  
Author(s):  
J. S. Abel ◽  
C. Berg ◽  
A. Geipel ◽  
U. Gembruch ◽  
U. Herberg ◽  
...  
Keyword(s):  
Prenatal Diagnosis ◽  
Health Status ◽  
Pulmonary Artery ◽  
Truncus Arteriosus ◽  
Intrauterine Fetal Death ◽  
Cardiac Anomaly ◽  
Chromosomal Anomalies ◽  
Cardiac Anomalies ◽  
Postnatal Outcome

Abstract Purpose To assess the spectrum of associated anomalies, the intrauterine course, postnatal outcome and management of fetuses with truncus arteriosus communis (TAC) Methods All cases of TAC diagnosed prenatally over a period of 8 years were retrospectively collected in two tertiary referral centers. All additional prenatal findings were assessed and correlated with the outcome. The accuracy of prenatal diagnosis was assessed. Results Thirty nine cases of TAC were diagnosed prenatally. Mean gestational age at first diagnosis was 22 weeks (range 13–38). Two cases were lost follow-up. Correct prenatal diagnosis of TAC was made in 87.5% and of TAC subtype in 90.5%. Prenatal diagnosis was incorrect in three cases: one newborn had aortic atresia with ventricular septal defect (VSD) postnatally, one had hypo-plastic right ventricle with dextro transposition of the great arteries (d-TGA) with coarctation of the aorta and a third newborn had tetralogy of fallot (TOF) with abnormal origin of the left pulmonary artery arising from the ascending aorta postnatally. These 3 cases were excluded from further analysis. In 26.5% of cases, TAC was an isolated finding. 38.2% of fetuses had additional chromosomal anomalies. Among them, microdeletion 22q11.2 was most common with a prevalence of 17.6% in our cohort. Another 3 fetuses were highly suspicious for non-chromosomal genetic syndromes due to their additional extra-cardiac anomalies, but molecular diagnosis could not be provided. Major cardiac and extra-cardiac anomalies occurred in between 8.8% and 58.8%, respectively. Predominantly, extra-cardiac anomalies occurred in association with chromosomal anomalies. Additionally, severe IUGR occurred in 17.6%. There were 14 terminations of pregnancy (41.2%), 1 (2.9%) intrauterine fetal death, 5 postnatal deaths (14.7%) and 14 (41.2%) infants were alive at last follow-up. Intention-to-treat survival rate was 70%. Mean follow-up among survivors was 42 months (range 6–104). Postoperative health status among survivors was excellent in 78.6%, but 46.2% needed repeated re-interventions due to recurrent pulmonary artery or conduit stenosis. The other 21.4% of survivors were significantly impaired due to non-cardiac problems. Conclusion Truncus arteriosus communis is a rare and complex cardiac anomaly that can be diagnosed prenatally with high precision. TAC is frequently associated with chromosomal and extra-cardiac anomalies, leading to a high intrauterine and postnatal loss rate due to terminations and perioperative mortality. Without severe extra-cardiac anomalies, postoperative health status is excellent, independent of the subtype of TAC, but the prevalence of repeated interventions due to recurrent stenosis is high.

scholarly journals Prenatal Diagnosis, Associated Findings and Postnatal Outcome of Fetuses With Truncus Arteriosus Communis (TAC)

2021 ◽  
Author(s):  
Judith S. Abel ◽  
Christoph Berg ◽  
Annegret Geipel ◽  
Ulrich Gembruch ◽  
Ulrike Herberg ◽  
...  
Keyword(s):  
Prenatal Diagnosis ◽  
Health Status ◽  
Pulmonary Artery ◽  
Truncus Arteriosus ◽  
Intrauterine Fetal Death ◽  
Cardiac Anomaly ◽  
Chromosomal Anomalies ◽  
Cardiac Anomalies ◽  
Postnatal Outcome

Abstract Purpose: To assess the spectrum of associated anomalies, the intrauterine course, postnatal outcome and management of fetuses with truncus arteriosus communis (TAC) Methods: All cases of TAC diagnosed prenatally over a period of 8 years were retrospectively collected in two tertiary referral centers. All additional prenatal findings were assessed and correlated with the outcome. The accuracy of prenatal diagnosis was assessed.Results: 39 cases of TAC were diagnosed prenatally. Mean gestational age at first diagnosis was 22 weeks (range, 13-38). Two cases were lost follow up. Correct prenatal diagnosis of TAC was made in 87.5% and of TAC subtype in 90.5%. Prenatal diagnosis was incorrect in three cases: One newborn had aortic atresia with ventricular septal defect (VSD) postnatally, one had hypoplastic right ventricle with dextro Transposition of the Great Arteries (d-TGA) with coartation of the aorta and a third newborn had Tetralogy of Fallot (TOF) with abnormal origin of the left pulmonary artery arising from the ascending aorta postnatally. These 3 cases were excluded from further analysis.In 26.5% of cases TAC was an isolated finding. 38.2% of fetuses had additional chromosomal anomalies. Among them, microdeletion 22q11.2 was most common with a prevalence of 17.6% in our cohort. Another 3 fetuses were highly suspicious for non-chromosomal genetic syndromes due to their additional extra-cardiac anomalies, but molecular diagnosis could not be provided. Major cardiac and extra-cardiac anomalies occurred in 8.8% and 58.8%, respectively. Predominantly, extra-cardiac anomalies occurred in association with chromosomal anomalies. Additionally, severe IUGR occurred in 17.6%. There were 14 terminations of pregnancy (41.2%), 1 (2.9%) intrauterine fetal death, 5 postnatal deaths (14.7%) and 14 (41.2%) infants were alive at last follow-up. Intention-to-treat survival rate was 70%. Mean follow up among survivors was 42 months (range, 6-104). Postoperative health status among survivors was excellent in 78.6%, but 46.2% needed repeated re-interventions due to recurrent pulmonary artery or conduit stenosis. The other 21.4% of survivors were significantly impaired due to non-cardiac problems.Conclusion: TAC is a rare and complex cardiac anomaly that can be diagnosed prenatally with high precision. TAC is frequently associated with chromosomal and extra-cardiac anomalies, leading to a high intrauterine and postnatal loss rate due to terminations and perioperative mortality. Without severe extra-cardiac anomalies, postoperative health status is excellent, independent of the subtype of TAC, but the prevalence of repeated interventions due to recurrent stenosis is high.

Prenatal Diagnosis and Postnatal Outcome of Fetuses with Pulmonary Atresia and Ventricular Septal Defect

2019 ◽  
Vol 41 (05) ◽  
pp. 514-525
Author(s):  
Ingo Gottschalk ◽  
Brigitte Strizek ◽  
Christel Jehle ◽  
Rüdiger Stressig ◽  
Ulrike Herberg ◽  
...  
Keyword(s):  
Ventricular Septal Defect ◽  
Septal Defect ◽  
Pulmonary Atresia ◽  
Intrauterine Fetal Death ◽  
Chromosomal Anomalies ◽  
Cardiac Anomalies ◽  
One Stage ◽  
Collateral Arteries ◽  
Postnatal Outcome

AbstractPurpose To assess the intrauterine course, associated conditions and postnatal outcome of fetuses with pulmonary atresia with ventricular septal defect (PAVSD).Methods All cases of PAVSD diagnosed prenatally over a period of 10 years with a minimum follow-up of 6.5 years were retrospectively collected in 3 tertiary referral centers.Results 50 cases of PAVSD were diagnosed prenatally. 44.0 % of fetuses had isolated PAVSD, 4.0 % had associated cardiac anomalies, 10.0 % had extra-cardiac anomalies, 38.0 % had chromosomal anomalies, 4.0 % had non-chromosomal syndromes. Among the 32 liveborn children, 56.3 % had reverse flow in the patent arterial duct, 25.0 % had major aortopulmonary collateral arteries (MAPCAs) with ductal agenesis and 18.7 % had a double supply. 17 pregnancies were terminated (34.0 %), there was 1 intrauterine fetal death (2.0 %), 1 neonatal death (2.0 %), and 6 deaths (12.0 %) in infancy. 25 of 30 (83.3 %) liveborn children with an intention to treat were alive at the latest follow-up. The mean follow-up among survivors was 10.0 years (range 6.5–15.1). 56.0 % of infants underwent staged repair, 44.0 % had one-stage complete repair. After exclusion of infants with additional chromosomal or syndromal anomalies, 88.9 % were healthy, and 11.1 % had mild limitations. The presence of MAPCAs did not differ significantly between survivors and non-survivors (p = 0.360), between one-stage or staged repair (p = 0.656) and healthy and impaired infants (p = 0.319).Conclusion The prognosis in cases without chromosomal or syndromal anomalies is good. MAPCAs did not influence prognosis or postoperative health. The incidence of repeat interventions due to recurrent stenoses is significantly higher after staged compared with single-stage repair.

scholarly journals Prenatal diagnosis, associated findings and postnatal outcome of fetuses with double outlet right ventricle (DORV) in a single center

2019 ◽  
Vol 47 (3) ◽  
pp. 354-364 ◽  
Author(s):  
Ingo Gottschalk ◽  
Judith S. Abel ◽  
Tina Menzel ◽  
Ulrike Herberg ◽  
Johannes Breuer ◽  
...  
Keyword(s):  
Prenatal Diagnosis ◽  
Right Ventricle ◽  
Single Ventricle ◽  
Double Outlet Right Ventricle ◽  
Biventricular Repair ◽  
Cardiac Anomalies ◽  
Postnatal Outcome ◽  
The Mean ◽  
Single Ventricle Palliation

Abstract Objective To assess the spectrum of associated anomalies, the intrauterine course, postnatal outcome and management of fetuses with double outlet right ventricle (DORV). Methods All cases of DORV diagnosed prenatally over a period of 8 years were retrospectively collected in a single tertiary referral center. All additional prenatal findings were assessed and correlated with the outcome. The accuracy of prenatal diagnosis was assessed. Results Forty-six cases of DORV were diagnosed prenatally. The mean gestational age at first diagnosis was 21+4 weeks (range, 13–37). A correct prenatal diagnosis of DORV was made in 96.3% of the cases. If the relation of the great arteries, the position of the ventricular septal defect (VSD) and additional cardiac anomalies are taken into account, the prenatal diagnosis was correct in 92.6% of the cases. One case was postnatally classified as transposition of the great arteries with subpulmonary VSD and was excluded from further analysis. A total of 41 (91.1%) fetuses with DORV had major additional cardiac anomalies, 30 (66.7%) had extracardiac anomalies and 13 (28.9%) had chromosomal or syndromal anomalies. Due to their complex additional anomalies, five (11.1%) of our 45 fetuses had multiple malformations and were highly suspicious for non-chromosomal genetic syndromes, although molecular diagnosis could not be provided. Disorders of laterality occurred in 10 (22.2%) fetuses. There were 17 terminations of pregnancy (37.8%), two (4.4%) intrauterine and seven (15.6%) postnatal deaths. Nineteen of 22 (86.4%) live-born children with an intention to treat were alive at last follow-up. The mean follow-up among survivors was 32 months (range, 2–72). Of 21 children who had already undergone postnatal surgery, eight (38.1%) achieved biventricular repair and 13 (61.9%) received univentricular palliation. One recently born child is still waiting for surgery. All children predicted prenatally to need a single ventricle palliation, and all children predicted to achieve biventricular repair, ultimately received the predicted type of surgery. After surgery, 14 of 18 (77.8%) children were healthy without any impairment. Conclusion DORV is a rare and often complex cardiac anomaly that can be diagnosed prenatally with high precision. DORV is frequently associated with major additional anomalies, leading to a high intrauterine and postnatal loss rate due to terminations or declined postnatal therapy. Without additional anomalies, the prognosis is good, although approximately 60% of children will have single ventricle palliation.

scholarly journals Prenatal diagnosis, associated findings and postnatal outcome in fetuses with congenitally corrected transposition of the great arteries

2020 ◽  
Author(s):  
Andrea Krummholz ◽  
I. Gottschalk ◽  
A. Geipel ◽  
U. Herberg ◽  
C. Berg ◽  
...  
Keyword(s):  
Prenatal Diagnosis ◽  
Fetal Echocardiography ◽  
Survival Rates ◽  
Cardiac Anomalies ◽  
Prenatal Counselling ◽  
Associated Malformations ◽  
Postnatal Outcome ◽  
Congenitally Corrected Transposition ◽  
Corrected Transposition

Abstract Purpose To analyze anatomic features and associated malformations in 37 prenatally detected cases of congenitally corrected transposition of the great arteries (ccTGA) and to evaluate the prenatal course, neonatal outcome and mid-term follow-up. Methods Retrospective analysis of prenatal ultrasound of 37 patients with ccTGA in two tertiary centers between 1999 and 2019. All fetuses received fetal echocardiography and a detailed anomaly scan. Postnatal outcome and follow-up data were retrieved from pediatric reports. Results Isolated ccTGA without associated cardiac anomalies was found in 13.5% (5/37), in all other fetuses additional defects such as VSD (73.0%), pulmonary obstruction (35.1%), tricuspid valve anomalies (18.9%), aortic arch anomalies (13.5%), ventricular hypoplasia (5.4%) or atrioventricular block (5.4%) were present. The rate of extracardiac malformations or chromosomal aberrations was low. There were 91.9% (34/37) live births and postnatal survival rates reached 91.2% in a mean follow-up time of 4.98 years. The prenatal diagnosis of ccTGA was confirmed postnatally in all but one documented live birth and the prenatal counselling regarding the expected treatment after birth (uni- versus biventricular repair) was reassured in the majority of cases. The postnatal intervention rate was high, 64.7% (22/34) received surgery, the intervention-free survival was 36.7%, 35.0% and 25.0% at 1 month, 1 year and 10 years, respectively. Conclusions ccTGA is a rare heart defect often associated with additional heterogeneous cardiac anomalies that can be diagnosed prenatally. The presented study demonstrates a favorable outcome in most cases but the majority of patients require surgical treatment early in life.

Digital solution for follow-up in congenital cardiac surgery

2021 ◽  
pp. 1-9
Author(s):  
Laura Carlson ◽  
Jacqueline O’Brien ◽  
Nitin Gujral ◽  
Vincent Chiang ◽  
Pedro del Nido ◽  
...  
Keyword(s):  
Cardiac Surgery ◽  
Health Status ◽  
Post Discharge ◽  
Web Based ◽  
Cardiac Anomalies ◽  
Children's Hospital ◽  
Congenital Cardiac Surgery ◽  
Phone Calls ◽  
Children’S Hospital

Abstract Background: In this era of public scrutiny, there is an ongoing need for innovative methods for patient follow-up. Objectives: As part of a quality initiative, we developed an automated post-operative follow-up system for patients following discharge after cardiac surgery at Boston Children’s Hospital. Methods: Discharge Communication (DisCo) is a web-based system developed at Boston Children’s Hospital. An automated text and e-mail with a link to a health status survey are sent at 30 days and 1 year post-discharge in English/Spanish. If there is no response, surveys are completed via phone calls to the patient/patient’s physician or chart review. Responses are stored in the DisCo database and the patient’s medical record. Patients who underwent cardiac surgery and survived to hospital discharge from October, 2016 received the surveys. Results: Overall, 3345 30-day and 2563 1-year surveys were sent between October, 2016 and June, 2020. Of 3345 30-day surveys, there were 3191 responses (95%). Of 2563 1-year surveys, there were 1807 responses (71%). Most patients/families responded directly to the link at 30 days (65% for paediatrics/75% for adults) and at 1 year (72% for paediatrics/78% for adults). Multi-variable logistic regression revealed that higher complexity of cardiac lesion, presence of major non-cardiac anomalies and presence of major residua were associated with readmission and catheter/surgical reinterventions. Non-cardiac anomalies were associated with increased need for services for learning, development or behaviour. Conclusions: DisCo provides a successful web-based health status assessment of patients following congenital cardiac surgery. It helps to identify high-risk patients who need closer follow-up.

scholarly journals Prenatal Detection of Cardiac Anomalies in Fetuses with Single Umbilical Artery: Diagnostic Accuracy Comparison of Maternal-Fetal-Medicine and Pediatric Cardiologist

2014 ◽  
Vol 2014 ◽  
pp. 1-8 ◽  
Author(s):  
Ilir Tasha ◽  
Rachel Brook ◽  
Heidi Frasure ◽  
Noam Lazebnik
Keyword(s):  
Umbilical Artery ◽  
Cardiac Anomaly ◽  
Inclusion Criteria ◽  
Single Umbilical Artery ◽  
Maternal Fetal Medicine ◽  
Fetal Medicine ◽  
Cardiac Anomalies ◽  
Accuracy Comparison ◽  
Good Agreement

Aim. To determine agreement of cardiac anomalies between maternal fetal medicine (MFM) physicians and pediatric cardiologists (PC) in fetuses with single umbilical artery (SUA).Methods. A retrospective review of all fetuses with SUA between 1999 and 2008. Subjects were studied by MFM and PC, delivered at our institution, and had confirmation of SUA and cardiac anomaly by antenatal and neonatal PC follow-up. Subjects were divided into four groups: isolated SUA, SUA and isolated cardiac anomaly, SUA and multiple anomalies without heart anomalies, and SUA and multiple malformations including cardiac anomaly.Results. 39,942 cases were studied between 1999 and 2008. In 376 of 39,942 cases (0.94%), SUA was diagnosed. Only 182 (48.4%) met inclusion criteria. Cardiac anomalies were found in 21% (38/182). Agreement between MFM physicians and PC in all groups combined was 94% (171/182) (95% CI [89.2, 96.8]). MFM physicians overdiagnosed cardiac anomalies in 4.4% (8/182). MFM physicians and PC failed to antenatally diagnose cardiac anomaly in the same two cases.Conclusions. Good agreement was noted between MFM physicians and PC in our institution. Studies performed antenatally by MFM physicians and PC are less likely to uncover the entire spectrum of cardiac abnormalities and thus neonatal follow-up is suggested.

Diagnosis, management, and results of treatment for aortopulmonary window

2004 ◽  
Vol 14 (5) ◽  
pp. 506-511 ◽  
Author(s):  
Murat Mert ◽  
Tufan Paker ◽  
Atif Akcevin ◽  
Gurkan Cetin ◽  
Ahmet Ozkara ◽  
...  
Keyword(s):  
Good Condition ◽  
Systolic Pressure ◽  
Ascending Aorta ◽  
Pulmonary Trunk ◽  
Cardiac Anomaly ◽  
Aortopulmonary Window ◽  
Congenital Cardiac Anomaly ◽  
Cardiac Anomalies ◽  
Transaortic Approach

The aortopulmonary window is a communication between the ascending aorta and the pulmonary trunk in the presence of two separate arterial valves. This uncommon congenital anomaly is reported rarely in the literature. We present here our experience with 16 patients, emphasizing the importance of early closure of the defect by a transaortic approach.We performed surgery on 16 patients over a period of 13 years using a transaortic approach under cardiopulmonary bypass. The median age of the patients at the time of operation was 6.5 months, with a range from 1 month to 11 years. Preoperative pulmonary arterial systolic pressure ranged from 30 to 100 mmHg. Associated cardiac anomalies were present in 7 of the patients, and were repaired at the same stage. The defect was between the ascending aorta and the proximal pulmonary trunk in 13 patients, and between the ascending aorta and the distal pulmonary trunk, with overriding of the orifice of the right pulmonary artery, in 3 patients. For closure, we used a patch of 0.4 mm Gore-Tex in 11, and gluteraldehyde-treated autologous pericardium in 5 of the patients.One patient died during surgery. The mean follow-up period for the surviving 15 patients was 52.2 months, with a range from 12 to 130 months. All the patients were in good condition during the follow-up, and no residual defects have been detected.Aortopulmonary window is a rare congenital cardiac anomaly, which can be repaired with very good operative results if surgery is performed before the development of irreversible pulmonary hypertension. We advise early correction of the defect with a transaortic patch, repairing all associated cardiac anomalies at the time of diagnosis.

scholarly journals Pregnancy follow-up and outcomes of patients diagnosed with fetal hypoplastic left heart syndrome

2020 ◽  
Vol 2 (2) ◽  
pp. 9-13
Author(s):  
Özge Yücel Çelik ◽  
Osman Yılmaz ◽  
Mehmet Obut ◽  
Ayşe Keleş ◽  
Mine Gültekin Çalık ◽  
...  
Keyword(s):  
Hypoplastic Left Heart Syndrome ◽  
Heart Diseases ◽  
Fetal Echocardiography ◽  
Cardiac Anomaly ◽  
Left Heart ◽  
Hypoplastic Left Heart ◽  
Cardiac Anomalies ◽  
Cardiac Evaluation ◽  
Left Heart Syndrome

Objective: The study aimed to evaluate the follow-up and outcomes of patients diagnosed with fetal hypoplastic left heart syndrome in the prenatal period. Materials: Between January 2017 and June 2019 the data of 36 patients diagnosed with fetal hypoplastic left heart syndrome (HLHS) in Etlik Zübeyde Hanım Woman’s Health Care Training and Research Hospital (EZH) were evaluated retrospectively. All patients who suspected for any fetal or cardiac anomaly were evaluated in detail ultrasound by two senior perinatologists and a pediatric cardiologist fort a detailed cardiac evaluation. The demographic characteristics, pregnancy follow-up and maternal and fetal outcomes of patients were evaluated. Results: During the study period fetal echocardiography was performed for 10.377 patients. A totally 382 (3.7%) fetuses were diagnosed with congenital heart diseases and 36 (9.4%) of these fetuses were diagnosed with hypoplastic left heart syndrome. Additional extracardiac anomalies were detected in 13 (36.1%) fetuses. Concomitant fetal cardiac anomalies were detected in 15 patients and ventricular septal defect (n: 11) and double outlet right ventricle (n:8) were the most common concomitant cardiac anomalies. Eight pregnancies with fetal HLHS were terminated. The data of 10 patient’s newborns were obtained and none of them lived up to a year.  Conclusion: The study revealed that the majority of patients with fetal HLHS are in low-risk population thus the routine fetal cardiac evaluation is of great importance. The prognosis and outcome of the pregnancy diagnosed with fetal HLHS are poor. The option of termination of the pregnancy should be considered. 

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