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The Journal of Maternal-fetal &... Dec 2022We retrospectively analyzed our center's experience with the prenatal diagnosis of isolated perimembranous ventricular septal defects that underwent primary surgical...
OBJECTIVE
We retrospectively analyzed our center's experience with the prenatal diagnosis of isolated perimembranous ventricular septal defects that underwent primary surgical repair in infancy.
METHODS
We identified patients born in Southern Nevada, between October 2012 and October 2020, with prenatal care that underwent surgical closure of an isolated large perimembranous ventricular septal defect between 1 and 12 months of age. The description at surgery defined ventricular septal defect morphology. We included only those with situs solitus, levocardia without dextroposition, and without any other cardiovascular abnormality. We analyzed prenatal detection rates for each of the eight years.
RESULTS
We identified 81 patients. Of the 81, 35 (43%) had trisomy 21. We identified no other aneuploidies in those that underwent surgical repair; however, 1 had a 15q13.3 deletion syndrome, and 1 had a 22 q11.2 deletion syndrome. Of the 81, 27 (33%) overall were prenatally diagnosed. Increasing prenatal detection rates strongly correlated with time ( = 0.92, = .002).
CONCLUSIONS
Trisomy 21 is common in isolated perimembranous ventricular septal defects undergoing primary repair in infancy. Further, prenatal detection rates significantly improved over time, up to 65% detection in the current years.
Topics: Pregnancy; Female; Humans; Retrospective Studies; Down Syndrome; Heart Septal Defects, Ventricular; Prenatal Diagnosis
PubMed: 34139939
DOI: 10.1080/14767058.2021.1940933 -
The Journal of Maternal-fetal &... Feb 2021To analyze the more recent outcomes of fetuses with different laterality defects. Out of 1508 fetuses with congenital heart disease (CHD) diagnosed by echocardiography...
To analyze the more recent outcomes of fetuses with different laterality defects. Out of 1508 fetuses with congenital heart disease (CHD) diagnosed by echocardiography between January 1995 and December 2015, 94 (6.3%) presented abnormal visceroatrial situs: 42 till December 2002 (group 1, analyzed retrospectively) and 52 since then until December 2015 (group 2, analyzed prospectively). Twenty-one had left isomerism (Lisom), 22 right isomerism (Risom), 16 situs inversus- dextrocardia (SVI-dx), 5 situs inversus- levocardia (SVI-levo), and 30 situs solitus-dextrocardia (SSol-dx). Seventy-six cases (81%) had CHD, more frequently complex atrioventricular defect. Eighteen cases showed normal heart: half of subgroups SVI-dx and SVI-levo, a third of SSol-dx. Postoperative mortality in continuing pregnancy in group 2 was 43.7 versus 40% in group 1, lower in SVs-dx (0 versus 50%) and Lisom (33.3 versus 66.7%), worse in Risom (71.4 versus 25%). Total mortality in group 2 was 48.3 versus 55% in group 1, better in Ssol-dx (37.5 versus 50%) and in Lisom (27.4 versus 71.4%). In isolated forms all but one case are alive. Our fetal cases presented a relevant mortality in both periods. The outcomes were slightly better in a more recent era in SSol and Lisom.What is known about this topic?Out of laterality defects, the heterotaxies (i.e. left and right isomerism) are known to be associated to a relevant mortality in fetal and neonatal cases.Heterotaxies have a variable position of the heart, but some authors evaluated only those with dextrocardia in their studies on different laterality defects.Controversial data are presented in the literature for the outcomes of fetuses with laterality defects. Many large studies analyzed the data of a very long time period in which it is opinable to compare the results of the treatment, because of a recent improved management and surgical techniques.What does this study add?Our study presents an experience of a single center with cases of different types of laterality defects observed during a routine fetal echocardiography, not only heterotaxies.The knowledge of different laterality defects and of their characteristics (association with cardiac and extracardiac anomalies or with normal heart) is useful for the prenatal counseling.We compare the postoperative and total mortality in more recent period (since 2003) with respect to the previous era (1995-2002).
Topics: Echocardiography; Female; Fetus; Heart Defects, Congenital; Heterotaxy Syndrome; Humans; Infant, Newborn; Pregnancy; Retrospective Studies; Situs Inversus
PubMed: 31106637
DOI: 10.1080/14767058.2019.1610737 -
Heart Asia 2018A 38-year-old woman, 37+3 weeker was incidentally detected to have fetal cardiomegaly during 36 weeks ultrasound and referred for fetal echocardiogram. Antenatal history...
UNLABELLED
A 38-year-old woman, 37+3 weeker was incidentally detected to have fetal cardiomegaly during 36 weeks ultrasound and referred for fetal echocardiogram. Antenatal history and anomaly scan were normal. Fetal echocardiogram showed heart rate of 153/min, sinus rhythm, situs solitus, levocardia, dilated right atrium and ventricle with venoatrial, atrioventricular and ventriculoarterial concordances. All cardiac valves were normal; cardiac crux was intact with separate mitral and tricuspid annuli (figure 1A-C and online supplementary video 1). Mild tricuspid regurgitation with peak velocity of 1.77 m/s was noted. There was no evidence of Ebstein's anomaly. Interventricular septum was intact. Pulmonary veins were seen entering left atrium (LA) without a retrocardiac chamber. Both aortic and ductal arches were adequate but with visualised in descending aorta. Three-vessel view showed (SVC) with normal spatial arrangement and sizes of aorta and pulmonary artery. Patent foramen ovale and patent ductus arteriosus were shunting normally. Inferior vena cava (IVC), hepatic and portal veins were normal. Significant hepatomegaly was seen without any evidence of hydrops. 10.1136/heartasia-2018-010998.supp1Supplementary data Figure 1(A) Fetal echocardiogram showing atrioventricular concordance with right atrium (RA) connected to morphological right ventricle and left atrium (LA) connected to morphological left ventricle (LV). Blue arrow depicts a large foramen ovale shunting from RA to LA. There is no retrocardiac chamber behind LA. (B) Three-vessel view in fetal echocardiogram showing normal right to left arrangement of superior vena cava (SVC), aorta (Ao) and pulmonary artery (PA). Interestingly, SVC is bigger than its neighbours which is against the norm. (C) Fetal short-axis echocardiographic view showing situs solitus with normal arrangement of RA and LA. RA is draining into right ventricle which is draining through PA into the ductal arch. However, significant aortic run-off is noted in aorta.
QUESTION
As per the available echocardiographic data, what is the most likely diagnosis for fetal heart failure?Idiopathic dilatation of SVC?Supracardiac total anomalous pulmonary venous connection?Fetal vein of Galen malformation?Fetal anaemia?
PubMed: 29636830
DOI: 10.1136/heartasia-2018-010998 -
Open Heart 2020Congenital pericardial defect (CPD) is a rare entity with an estimated frequency of 0.01%-0.04%. The recognition of this anomaly is important since it can be associated...
OBJECTIVE
Congenital pericardial defect (CPD) is a rare entity with an estimated frequency of 0.01%-0.04%. The recognition of this anomaly is important since it can be associated with serious complications. The aim of this study and review was to describe clinical and imaging features that help in establishing the diagnosis of this condition.
METHODS
We retrospectively reviewed all adult patients at the Cleveland Clinic Health System with the diagnosis of CPD between the years 2000 and 2015. Baseline clinical characteristics, clinical manifestations, ECG, transthoracic echocardiography (TTE), cardiac CT and cardiac magnetic resonance (CMR) images were reviewed.
RESULTS
Eight patients were included in the study. Sixty-three percent of patients were males with mean age at diagnosis of 48 years, 63% had a partial pericardial defect on the left side and right ventricular (RV) dilation on TTE. Three patients had CMR. Levocardia was present in all CMRs. One patient had greater than 60° clockwise rotation and none of the CMRs showed ballooning of the left ventricular apex. One patient required surgical pericardioplasty. The remaining seven patients had a median follow-up of 17.3 months (5-144.9 months) and all remained asymptomatic.
CONCLUSION
CPDs are more likely to be partial on the left side and patients often have RV dilation on the TTE and levocardia on CMR. Most patients remain stable and do not require surgical intervention. TTE and CMR play an important role in making the diagnosis of this anomaly.
Topics: Adult; Cardiac Imaging Techniques; Clinical Decision-Making; Critical Pathways; Decision Support Techniques; Echocardiography; Electrocardiography; Female; Heart Defects, Congenital; Humans; Magnetic Resonance Imaging; Male; Middle Aged; Pericardium; Predictive Value of Tests; Prognosis; Retrospective Studies; Time Factors; Tomography, X-Ray Computed
PubMed: 32076559
DOI: 10.1136/openhrt-2019-001103 -
European Heart Journal Apr 2017
Topics: Diverticulum; Heart Diseases; Heart Septal Defects, Atrial; Heart Ventricles; Humans; Infant, Newborn; Levocardia; Magnetic Resonance Angiography; Male; Multimodal Imaging; Tomography, X-Ray Computed
PubMed: 28028007
DOI: 10.1093/eurheartj/ehw519 -
Journal of Community Hospital Internal... 2019Tetralogy of Fallot is the most common cyanotic congenital heart disease. It consists of right ventricular outflow tract obstruction, a ventricular septal defect,...
Tetralogy of Fallot is the most common cyanotic congenital heart disease. It consists of right ventricular outflow tract obstruction, a ventricular septal defect, abnormally located aorta and right ventricular hypertrophy. It usually occurs as an isolated anomaly with a normally placed heart and abdominal viscera. We present a case of a 19 years old female who presented with a prolonged history of shortness of breath (SOB) and cyanosis. After undergoing echocardiography and cardiac computed tomography angiogram (CTA), she was diagnosed to be a case of Tetrology of Fallot (TOF) in association with situs inversus with levocardia also termed 'isolated levocardia'. The patient underwent surgical correction and she was asymptomatic with no residual cardiac defects on follow up after 6 months. Isolated levocardia is a rare condition that is usually associated with severe cardiac defects and a low life expectancy in untreated patients. It is unusual for it to be diagnosed in adults as in our case.
PubMed: 31258874
DOI: 10.1080/20009666.2019.1601980 -
Journal of Cardiovascular Echography 2020A newborn without prenatal diagnosis, with bronchial and abdominal situs inversus in levocardia, was referred to our hospital for accurate evaluation; echocardiography...
A newborn without prenatal diagnosis, with bronchial and abdominal situs inversus in levocardia, was referred to our hospital for accurate evaluation; echocardiography showed venoatrial connections in mirror-image arrangement, atrioventricular (AV) discordance, and double-outlet right ventricle (DORV). Additional cardiac malformations were double upper caval district, atrial communication, subpulmonary interventricular communication, and moderate subvalvular and valvular pulmonary stenosis. Few days after birth, the patient presented low oxygen saturation and the heart team decided for a palliative surgery. We describe a very rare case in a newborn with bronchial-abdominal mirror imagery, AV discordance, and DORV in levocardia.
PubMed: 33828947
DOI: 10.4103/jcecho.jcecho_65_20 -
European Heart Journal. Case Reports Jul 2021Cryoballoon-based pulmonary vein isolation (cbPVI) is a standardized treatment of atrial fibrillation. In complex anatomies, radiofrequency ablation (rfPVI) is usually...
BACKGROUND
Cryoballoon-based pulmonary vein isolation (cbPVI) is a standardized treatment of atrial fibrillation. In complex anatomies, radiofrequency ablation (rfPVI) is usually preferred. We describe the first cbPVI in a rare patient with SI and levocardia.
CASE SUMMARY
A 41-year-old male patient with paroxysmal atrial fibrillation was referred to our clinic after a previous, unsuccessful cbPVI procedure. Observation of an atypical lead-wire position due to an abnormal anatomy of the inferior vena cava led to its initial termination. A subsequent thoraco-abdominal computed tomography revealed situs inversus abdominalis and levocardia and the procedure was re-attempted in our clinic. Transseptal puncture (TSP) was guided via transoesophageal echocardiography and fluoroscopy, using a SL0-Sheath and a standard BRK-needle. Advancement of the sheath initially failed but after additional dilatation with an Inoue dilator, transseptal passage of the sheath was successful. Due to the unusual antero-cranial TSP, the septal pulmonary veins (PV) contrasted poorly. After repeat TSP, a steerable FlexCath Advance sheath was introduced into the left atrium using an Amplatz Super Stiff guidewire. Subsequently, all PV were intubated with the Achieve catheter, over which a 2nd generation cryoballoon was introduced. Despite the practical challenges in this case, all PV were isolated.
DISCUSSION
The main challenges include the achievement of transseptal access and manipulation of the cryoballoon to achieve a patent seal of the pulmonary veins. cbPVI eliminates the need for constant re-positioning of the ablation catheter and might facilitate the creation of durable lesions under such difficult anatomical conditions.
PubMed: 34240004
DOI: 10.1093/ehjcr/ytab245 -
Journal of Surgical Case Reports Jul 2022Situs inversus (SI) is a very rare congenital disease affecting one in 10 000 people. It is characterized by a mirror image transposition of both abdominal and thoracic...
Situs inversus (SI) is a very rare congenital disease affecting one in 10 000 people. It is characterized by a mirror image transposition of both abdominal and thoracic organs. Diagnosis of SI is usually made incidentally while investigating for unrelated medical problem. It can be associated with cardiac and respiratory anomalies that may cause perioperative morbidity if not diagnosed before surgery. There are limited case reports in literature of SI patients that underwent bariatric procedures. We report a case of a 32-year-old female with SI partialis and levocardia who successfully underwent laparoscopic sleeve gastrectomy. We concluded that sleeve gastrectomy is safe in patients with SI if diagnosed preoperatively and if all the necessary precautions are taken into consideration before and during the surgery.
PubMed: 35919692
DOI: 10.1093/jscr/rjac325 -
Journal of Cardiology May 2017The right ventricle has a proclivity to wrap around the left ventricle outflow tract (LVOT) in congenitally corrected transposition (CCT) patients with apicocaval...
BACKGROUND
The right ventricle has a proclivity to wrap around the left ventricle outflow tract (LVOT) in congenitally corrected transposition (CCT) patients with apicocaval ipsilaterality, which may influence the outcome of the double switch operation (DSO). The goal of this study was to determine if the LVOT is compressed by the right ventricle in this setting.
METHODS
A total of 103 patients with CCT were divided into four groups according to ventricular looping and apical position, including Group A (D-loop and levocardia), Group B (L-loop and dextrocardia), Group C (D-loop and dextrocardia), and Group D (L-loop and levocardia). Computed tomography was used to define left-right laterality and ventro-dorsal relationship of the LVOT.
RESULTS
Apicocaval ipsilaterality was found in 57 patients (Group A, n=25; Group B, n=32), in whom the right ventricle was found to wrap around the LVOT. Among them, 49 (86%) had LVOT obstruction. In 46 patients without apicocaval ipsilaterality (Group C, n=10; Group D, n=36), 31 had LVOT obstruction (67.4%). LVOT obstruction was more prone to occur in patients with apicocaval ipsilaterality compared with those without (p=0.025), and was more significant in the situs solitus (p=0.058) than in situs inversus (p=0.547).
CONCLUSIONS
LVOT obstruction was prone to occur in CCT patients with situs solitus and apicocaval ipsilaterality (Group B). The ventricular outflow patency was influenced by apical position, which should be considered to avoid a posterior ventricular outflow tract from compression after DSO.
Topics: Adolescent; Child; Female; Heart Ventricles; Humans; Male; Retrospective Studies; Situs Inversus; Transposition of Great Vessels; Ventricular Outflow Obstruction
PubMed: 27842759
DOI: 10.1016/j.jjcc.2016.07.019