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.

Anatomic correction of congenitally corrected transposition and its close cousins

2006 ◽  
Vol 16 (S3) ◽  
pp. 85-90 ◽  
Author(s):  
Edward L. Bove ◽  
Richard G. Ohye ◽  
Eric J. Devaney ◽  
Hiromi Kurosawa ◽  
Toshiharu Shin'oka ◽  
...  
Keyword(s):  
Surgical Approaches ◽  
Long Term Effects ◽  
The Past ◽  
Congenital Cardiac Malformation ◽  
Associated Malformations ◽  
Atrioventricular Discordance ◽  
Congenitally Corrected Transposition ◽  
Corrected Transposition

The congenital cardiac malformation characterized by discordant connections between the atriums and ventricles, as well as those between the ventricles and the arterial trunks, has been given many names. The terms atrioventricular discordance, l-transposition of the great arteries, ventricular inversion, and congenitally corrected transposition have all been used. Regardless of terminology, this complex congenital anomaly has only recently been studied to analyze the long-term effects of its natural history and outcomes following traditional surgical repair of the associated malformations which serve to uncorrect the circulatory pathways. As more patients survive into adulthood, the effects of this condition are now better understood, and the surgical approaches used in the past are being re-examined in light of longer-term follow up.

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.

Anatomic correction of congenitally corrected transposition and its close cousins

2006 ◽  
Vol 16 (S3) ◽  
pp. 85-90
Author(s):  
Edward L. Bove ◽  
Richard G. Ohye ◽  
Eric J. Devaney ◽  
Hiromi Kurosawa ◽  
Toshiharu Shin'oka ◽  
...  
Keyword(s):  
Surgical Approaches ◽  
Long Term Effects ◽  
The Past ◽  
Congenital Cardiac Malformation ◽  
Associated Malformations ◽  
Atrioventricular Discordance ◽  
Congenitally Corrected Transposition ◽  
Corrected Transposition

The congenital cardiac malformation characterized by discordant connections between the atriums and ventricles, as well as those between the ventricles and the arterial trunks, has been given many names. The terms atrioventricular discordance, l-transposition of the great arteries, ventricular inversion, and congenitally corrected transposition have all been used. Regardless of terminology, this complex congenital anomaly has only recently been studied to analyze the long-term effects of its natural history and outcomes following traditional surgical repair of the associated malformations which serve to uncorrect the circulatory pathways. As more patients survive into adulthood, the effects of this condition are now better understood, and the surgical approaches used in the past are being re-examined in light of longer-term follow up.

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.

scholarly journals Prenatally Diagnosis and Outcome of Fetuses with Cardiac Rhabdomyoma - Single Centre Experience

2017 ◽  
Vol 5 (2) ◽  
pp. 193-196 ◽  
Author(s):  
Ramush Bejiqi ◽  
Ragip Retkoceri ◽  
Hana Bejiqi
Keyword(s):  
Prenatal Diagnosis ◽  
Tuberous Sclerosis ◽  
Fetal Echocardiography ◽  
Tumour Size ◽  
Primary Tumour ◽  
Postnatal Period ◽  
Single Centre ◽  
Cardiac Rhabdomyoma ◽  
Ultrasound Evaluation

BACKGROUND: Cardiac rhabdomyoma (CRs) are the most common primary tumour of the heart in infants and children. Usually are multiple and, basing on the location can cause a haemodynamic disturbance, dysrhythmias or heart failure during the fetal and early postnatal period. CRs have a natural history of spontaneous regression and are closely associated with tuberous sclerosis complex (TSC). It has an association with tuberous sclerosis (TS), and in those, the tumour may regress and disappear completely, or remain consistent in size. AIM: We aimed to evaluate the prenatal diagnosis, clinical presentation and outcome of CRs and their association with TSC in a single centre. The median follow-up period was three years (range: 6 months - 5 years). MATERIAL AND METHODS: We reviewed medical records of all fetuses diagnosed prenatally with cardiac rhabdomyoma covering the period January 2010 to December 2016 which had undergone detailed ultrasound evaluation at a single centre with limited technical resources. RESULTS: Twelve fetuses were included in the study; mostly had multiple tumours and a total of 53 tumours were identified in all patients - the maximum was one fetus with16 tumours. All patients were diagnosed prenatally by fetal echocardiography. In two patient's haemodynamic disturbances during the fetal period was noted and pregnancies have been terminated. After long consultation termination of pregnancy was chosen by the parents in totally 8 cases. In four continuing pregnancies during the first year of live tumours regressed. TSC was diagnosed in all patients during the follow-up. CONCLUSIONS: Cardiac rhabdomyoma are benign from the cardiovascular standpoint in most affected fetuses. An early prenatal diagnosis may help for an adequate planning of perinatal monitoring and treatment with the involvement of a multidisciplinary team. Large tumour size, the number of tumours and localisation may cause hydrops, and they are significantly associated with poor neonatal outcome.

scholarly journals Effectiveness of Bidirectional Glenn Shunt Placement for Palliation in Complex Congenitally Corrected Transposed Great Arteries

2020 ◽  
Vol 47 (1) ◽  
pp. 15-22
Author(s):  
Kai Ma ◽  
Lei Qi ◽  
Zhongdong Hua ◽  
Keming Yang ◽  
Hao Zhang ◽  
...  
Keyword(s):  
Glenn Shunt ◽  
Shunt Placement ◽  
Anatomic Repair ◽  
Bidirectional Glenn ◽  
Palliative Procedure ◽  
Bidirectional Glenn Shunt ◽  
Group 3 ◽  
Congenitally Corrected Transposition ◽  
Corrected Transposition

Surgery for complex congenitally corrected transposed great arteries is one of the greatest challenges in cardiovascular surgery. We report our experience with bidirectional Glenn shunt placement as a palliative procedure for complex congenitally corrected transposition. We retrospectively identified 50 consecutive patients who had been diagnosed with congenitally corrected transposition accompanied by left ventricular outflow tract obstruction and ventricular septal defect and who had then undergone palliative bidirectional Glenn shunt placement at our institution from January 2005 through December 2014. Patients were divided into 3 groups according to subsequent surgeries: Fontan completion (total cavopulmonary connection, 13 patients) (group 1), anatomic repair (hemi-Mustard and Rastelli procedures without Glenn takedown, 11 patients) (group 2), and prolonged palliation (no further surgery, 26 patients) (group 3). After shunt placement, no patient died or had ventricular dysfunction. Overall, mean oxygen saturation increased significantly from 79.5% ± 13.5% preoperatively to 94.1% ± 7.3% (P <0.001). The median time from shunt placement to Fontan completion and anatomic repair, respectively, was 2.1 years (range, 1.6–5.2 yr) and 1.1 years (range, 0.6–2.4 yr). Only 2 late deaths occurred, both in group 1. In group 3, time from shunt placement to latest follow-up was 4.5 years (range, 2.3–8 yr). At latest follow-up, mean oxygen saturation was 91.6% ± 10.3%, and no patients had impaired ventricular function. Bidirectional Glenn shunt placement as an optional palliative procedure for complex congenitally corrected transposition has favorable outcomes. Later, patients can feasibly be treated by Fontan completion or anatomic repair. Use of a bidirectional Glenn shunt for open-ended palliation is also acceptable.

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