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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 -
The British Journal of Radiology Oct 2019Viscero-atrial situs encompasses the laterality, relative position and configuration of the abdominal viscera, the atria of the heart and the tracheobronchial tree....
Viscero-atrial situs encompasses the laterality, relative position and configuration of the abdominal viscera, the atria of the heart and the tracheobronchial tree. Determining the situs and cardiac position is the first step in the commonly used sequential, segmental approach to the imaging evaluation of congenital heart defects (CHD). Abnormalities of visceroatrial situs and cardiac position are frequently associated with the presence of complex CHDs and accurate assessment of situs abnormalities can help predict the probability and type of the defect. Multidetector CT (MDCT) angiography, with its multiplanar reformatting and volume rendering techniques, offers accurate information about the morphology and three-dimensional relationships of the various cardiac and extra cardiac structures. In this pictorial essay, we present the MDCT imaging findings of the spectrum of abnormalities of visceroatrial situs and cardiac position, using a third generation dual source CT scanner.
Topics: Bronchi; Computed Tomography Angiography; Heart; Heart Atria; Heart Defects, Congenital; Humans; Levocardia; Liver; Multidetector Computed Tomography; Stomach; Trachea; Viscera
PubMed: 31271542
DOI: 10.1259/bjr.20190231 -
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 -
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 -
Antenatal ultrasound diagnosis of small bowel non-rotation in complex left isomerism: a case report.International Journal of Surgery Case... 2019A multiparous expectant mother was referred to our tertiary unit at 23 weeks with a complex fetal cardiac anomaly in the context of suspected heterotaxy syndrome. The...
PRESENTATION OF CASE
A multiparous expectant mother was referred to our tertiary unit at 23 weeks with a complex fetal cardiac anomaly in the context of suspected heterotaxy syndrome. The cardiac findings were consistent with isomerism: the fetal cardiac position was levocardia with a single functioning double outlet ventricle and AV valve, pulmonary stenosis, and interrupted inferior vena cava (IVC) with azygous continuation. The fetal abdominal situs was also altered, with the stomach to the right, and the hepatobiliary system midline to left. The spleen was not identified antenatally or postnatally. At 36 weeks, ultrasound revealed an abnormal bowel pattern with small bowel loops on the right side of the abdomen and large bowel on the left, suggesting a diagnosis of non- rotation. The infant was delivered vaginally at 39 weeks. The cardiac diagnosis and non-rotation of the small bowel were confirmed by postnatal echocardiography and contrast fluoroscopy.
DISCUSSION
Heterotaxy syndrome is traditionally classified into right or left isomerism depending on how and where the organs are anatomically arranged. The case presented here demonstrates mixed laterality and prenatal ultrasound features of non-rotation.
CONCLUSION
It is important to be informed of the embryological variants of isomerism and actively seek antenatal evidence of bowel non-rotation in such cases.
PubMed: 30822676
DOI: 10.1016/j.ijscr.2018.11.069 -
Journal of Arrhythmia Dec 2018Implantation of a cardiac resynchronization therapy (CRT) device in patients with congenital heart disease may be challenging because of the particular underlying...
Implantation of a cardiac resynchronization therapy (CRT) device in patients with congenital heart disease may be challenging because of the particular underlying anatomy. We present the case of a right-sided transvenous implantation of a dual-chamber CRT-D device in a patient with situs inversus totalis and mirror image dextrocardia. To facilitate our anatomic orientation and to overcome unusual hand-eye coordination problems, we decided to flip the fluoroscopic image projection by 180° (right-left), creating the optical impression of a "normalized" heart position (levocardia). This simple approach allowed us to successfully implant the device using a conventional left-sided CRT delivery system.
PubMed: 30555613
DOI: 10.1002/joa3.12120 -
Congenital Heart Disease Jul 2018We reviewed all patients evaluated at our center with situs solitus, levocardia, and a right aortic arch that were born between January 2000 and January 2018. (Observational Study)
Observational Study
PATIENTS
We reviewed all patients evaluated at our center with situs solitus, levocardia, and a right aortic arch that were born between January 2000 and January 2018.
RESULTS
From our databases, we identified 204 patients. We excluded patients with a double aortic arch from analysis. Of the 204 patients, 103 (50%) were male. Of the 204 patients, 95 (47%) had an isolated right aortic arch. Of the 95 with an isolated right aortic arch, 4 (4%) had chromosome 22q11.2 deletion syndrome, and 89 (94%) had a vascular ring. Of the 204 patients, 109 (53%) had a surgical intracardiac malformation. Of the 109, with an associated surgical intracardiac malformation, 38 (35%) had chromosome 22q11.2 deletion syndrome, and 28 (26%) had a vascular ring. Overall, of the 204 patients, 200 (98%) had a vascular ring, an associated intracardiac malformation, or both. Prenatal detection was 85% (40/47) for the subset of patients born in Southern Nevada between January 2015 and January 2018.
CONCLUSION
this review, to best of our knowledge, reports one of largest series of right aortic arches in the literature. Data from our cohort demonstrates that a right aortic arch in situs solitus is almost always associated with pathology. Further, our center's right aortic arch prenatal detection rate exceeds previous reports.
Topics: Abnormalities, Multiple; Aorta, Thoracic; Diagnosis, Differential; Echocardiography; Female; Humans; Infant, Newborn; Magnetic Resonance Imaging, Cine; Male; Situs Inversus; Tomography, X-Ray Computed; Vascular Malformations
PubMed: 30033669
DOI: 10.1111/chd.12623 -
Archives of Disease in Childhood.... Apr 2019
Topics: Duodenal Obstruction; Female; Humans; Infant, Newborn; Intestinal Atresia; Levocardia; Radiography; Situs Inversus
PubMed: 29703815
DOI: 10.1136/archdischild-2018-314803 -
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 -
Surgical Case Reports Feb 2018Situs inversus is a rare congenital condition that is currently classified into two types: complete situs inversus (situs inversus totalis, SIT) and partial situs...
BACKGROUND
Situs inversus is a rare congenital condition that is currently classified into two types: complete situs inversus (situs inversus totalis, SIT) and partial situs inversus (situs inversus partialis, SIP). In SIP patients, some organs are inverted and others are in their expected position, and individual patient variation in organ position increases surgical difficulty. Several surgeons have performed laparoscopic or robotic surgeries in situs inversus patients, but almost all were SIT patients. We report the first case, to our knowledge, of an SIP patient with gastric cancer who was successfully treated by robot-assisted distal gastrectomy (RADG) with lymph node dissection.
CASE PRESENTATION
A 64-year-old woman diagnosed with early gastric cancer on the posterior midbody of the stomach was referred to our hospital for treatment. Computed tomography showed levocardia and inverted abdominal organs without enlarged lymph nodes or distant metastases. Polysplenia syndrome, intestinal malrotation, and left-sided gallbladder were also detected. RADG with D1+ lymph node dissection and Billroth I reconstruction (delta-shaped anastomosis) were performed using robotics. Hepatopathy caused by a liver retractor and pancreatic fistula were identified during the postoperative course, and the latter was classified as grade II based on Clavien-Dindo classification. The patient was discharged 18 days after the operation.
CONCLUSIONS
Preoperative three-dimensional imaging is beneficial, and anatomical organ identification should be routinely performed, especially in SIP patients. We consider RADG a therapeutic option in SIP patients.
PubMed: 29441475
DOI: 10.1186/s40792-018-0422-7