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Animals : An Open Access Journal From... Jun 2023Developmental anomalies are an important cause of stillbirth and early perinatal death in companion animals. Many of these disorders remain poorly understood and provide...
Developmental anomalies are an important cause of stillbirth and early perinatal death in companion animals. Many of these disorders remain poorly understood and provide an opportunity as a spontaneous animal model for human disease. Pentalogy of Cantrell is a rare congenital syndrome described in human neonates. It is a ventral midline closure defect with a proposed familial inheritance in humans. This syndrome involves five defects, including the thoracoabdominal wall, sternal, diaphragmatic, pericardial and cardiac malformations. Diverse expressions of these defects have been described in humans and sporadically in domestic animals. This severe syndrome commonly harbors a poor prognosis, posing an ethical and surgical dilemma. To better understand this syndrome and its presentation in dogs, we describe two rare cases of Pentalogy of Cantrell in a litter of papillon dogs. The affected puppies had anomalies compatible with the Pentalogy of Cantrell, including thoracoabdominal schisis, ectopia cordis, sternal cleft, pericardial agenesis, and diaphragmatic defects. The diagnosis was confirmed by advanced imaging (computed tomography) and postmortem examinations. The family history of this litter was explored and other cases in domestic animals were reviewed. This is the first report of the complete Pentalogy of Cantrell with ectopia cordis in the dog and the only report on papillons. Similar to human cases, possible familial inheritance and suspected male gender bias were observed. Further research on this novel animal model, its pathogenesis and its hereditary basis, may be helpful in better understanding this rare developmental disorder.
PubMed: 37443889
DOI: 10.3390/ani13132091 -
Annals of Medicine and Surgery (2012) Jul 2023Ectopia cordis is a rare type of malformation where the heart is not located normally. It may be partially or completely located outside the thoracic cavity and can be...
UNLABELLED
Ectopia cordis is a rare type of malformation where the heart is not located normally. It may be partially or completely located outside the thoracic cavity and can be associated with other congenital abnormalities.
CASE PRESENTATION
This case report is of a 34-week- and 6-day-old female fetus with a birth weight of 2040 g, a height of 41 cm, and a head circumference of 32 cm. The initial physical examination revealed a responsive newborn with an externalized heart outside the chest, with pericardium protection. In addition, a thoracic wall defect was detected suggesting incomplete formation of the septum bone. Moreover, in this case, the echocardiography report showed a multiple ventricular septal defect.
CONCLUSION
The management of ectopia cordis is a challenge for any obstetrician and pediatric surgeon due to its rarity. It causes mental agony and anxiety to the parents. With an early diagnosis, termination of pregnancy can be one of the options. Once it is diagnosed late, it needs a multidisciplinary approach, and the services of a very experienced pediatric surgeon to improve the prognosis.
PubMed: 37427174
DOI: 10.1097/MS9.0000000000000329 -
Journal of Cardiovascular Echography 2023A 48-year-old man with AIDS/HIV and a previous history of tuberculous pericarditis 25 years earlier was admitted to our department of infectious diseases because of P....
A 48-year-old man with AIDS/HIV and a previous history of tuberculous pericarditis 25 years earlier was admitted to our department of infectious diseases because of P. carinii pneumonia. A CT (computed tomography) scan described diffuse pericardial thickening with extensive pericardial calcifications on both ventricles. A transthoracic echocardiogram showed all the typical hemodynamic features of pericardial constriction. A review of the CT with 3D reconstruction revealed the presence of ring-shaped pericardial calcification at the basal segments of right and left ventricles extending over the inferior atrioventricular groove, the inferior interventricular groove and the cranial wall of the right atrium. Only a few cases of ring-shaped constrictive pericarditis have been reported and both global ventricular constriction and localized segmental constriction have been described. Our case wants to underline how important a comprehensive multi-modality imaging approach is to this rare form of constrictive pericarditis.
PubMed: 37426717
DOI: 10.4103/jcecho.jcecho_23_22 -
The American Journal of Case Reports Jul 2023BACKGROUND Cardiac perforation is a rare complication of cardiac implantable electronic devices, with a reported incidence ranging from 0.1% to 5.2%. Delayed...
BACKGROUND Cardiac perforation is a rare complication of cardiac implantable electronic devices, with a reported incidence ranging from 0.1% to 5.2%. Delayed perforation, defined as perforation occurring more than 1 month after implantation, is more uncommon. In this report, we present a case of cardiac perforation involving the right ventricle wall that occurred 9 years after pacemaker implantation. CASE REPORT A 79-year-old woman presented with symptoms of dyspnea and was subsequently admitted to a hospital. She had undergone pacemaker implantation for a complete atrioventricular block 9 years prior to the presentation. The patient had right ventricular failure to capture and a resultant complete atrioventricular block. Computed tomography imaging revealed that the right ventricular lead had clearly protruded outside the heart; however, no pericardial effusion was observed. When the patient underwent open surgical repair, the ventricular tined lead was noted to be traversing the right ventricular apex. Device interrogation revealed a sudden increase followed by a gradual decrease in the right ventricular pacing threshold over the course of 2 months, indicating that the lead had slowly traversed the right ventricular muscle before ultimately rupturing through it. CONCLUSIONS This study described the case of a delayed right ventricular pacemaker lead perforation that occurred 9 years after implantation, which was managed via open surgical repair.
Topics: Female; Humans; Aged; Heart Ventricles; Atrioventricular Block; Pacemaker, Artificial; Pericardial Effusion; Heart Injuries
PubMed: 37401051
DOI: 10.12659/AJCR.940291 -
Journal of Vascular Surgery Cases and... Sep 2023A 69-year-old patient presented with a 9-month history of constitutional symptoms and a 3-week history of increasing abdominal and back pain. He had a history of...
A 69-year-old patient presented with a 9-month history of constitutional symptoms and a 3-week history of increasing abdominal and back pain. He had a history of bacillus Calmette-Guérin immunotherapy for bladder cancer 9 months earlier. An infrarenal mycotic aneurysm was detected by positron emission tomography-computed tomography. His abdominal aorta was reconstructed using a tube graft tailored from a bovine pericardium sheet. We chose this graft because of its acellular nature and reduced risk of postoperative infection. The culture from the aortic wall yielded acid fast bacilli, and he was treated with antituberculosis medication. His postoperative recovery was uneventful, except for chylous ascites.
PubMed: 37388673
DOI: 10.1016/j.jvscit.2023.101213 -
Europace : European Pacing,... Jul 2023Pulsed field ablation (PFA) is a new, non-thermal ablation modality for pulmonary vein (PV) isolation in patients with atrial fibrillation (AF). The multi-centre...
AIMS
Pulsed field ablation (PFA) is a new, non-thermal ablation modality for pulmonary vein (PV) isolation in patients with atrial fibrillation (AF). The multi-centre EUropean Real World Outcomes with Pulsed Field AblatiOn in Patients with Symptomatic AtRIAl Fibrillation (EU-PORIA) registry sought to determine the safety, efficacy, and learning curve characteristics for the pentaspline, multi-electrode PFA catheter.
METHODS AND RESULTS
All-comer AF patients from seven high-volume centres were consecutively enrolled. Procedural and follow-up data were collected. Learning curve effects were analysed by operator ablation experience and primary ablation modality. In total, 1233 patients (61% male, mean age 66 ± 11years, 60% paroxysmal AF) were treated by 42 operators. In 169 patients (14%), additional lesions outside the PVs were performed, most commonly at the posterior wall (n = 127). Median procedure and fluoroscopy times were 58 (interquartile range: 40-87) and 14 (9-21) min, respectively, with no differences due to operator experience. Major complications occurred in 21/1233 procedures (1.7%) including pericardial tamponade (14; 1.1%) and transient ischaemic attack or stroke (n = 7; 0.6%), of which one was fatal. Prior cryoballoon users had less complication. At a median follow-up of 365 (323-386) days, the Kaplan-Meier estimate of arrhythmia-free survival was 74% (80% for paroxysmal and 66% for persistent AF). Freedom from arrhythmia was not influenced by operator experience. In 149 (12%) patients, a repeat procedure was performed due to AF recurrence and 418/584 (72%) PVs were durably isolated.
CONCLUSION
The EU-PORIA registry demonstrates a high single-procedure success rate with an excellent safety profile and short procedure times in a real-world, all-comer AF patient population.
Topics: Humans; Male; Middle Aged; Aged; Female; Atrial Fibrillation; Poria; Treatment Outcome; Catheter Ablation; Fluoroscopy; Pulmonary Veins; Recurrence
PubMed: 37379528
DOI: 10.1093/europace/euad185 -
The Indian Journal of Radiology &... Jul 2023Paragangliomas are neuroendocrine tumors of the sympathetic and parasympathetic nervous system that originate from neural crest cells. Less than 1% of paragangliomas are...
Paragangliomas are neuroendocrine tumors of the sympathetic and parasympathetic nervous system that originate from neural crest cells. Less than 1% of paragangliomas are found in the heart, originating from intrinsic cardiac ganglia cells in the posterior wall the atria, atrioventricular groove, and along the root of the great vessels. A 10-year review of our institution's database identified nine patients who had documented intrapericardial paragangliomas. We describe the multimodality imaging appearance of these tumors. The most common findings include embedment and wrapping around the great vessels and atrioventricular groove within the confines of the pericardium, markedly avid heterogeneous enhancement, distinct engorged neovascularization, and in large lesions, central low attenuation areas compatible with hemorrhage, necrosis, or cystic degeneration.
PubMed: 37362356
DOI: 10.1055/s-0041-1741092 -
Journal of Cardiology Cases Jul 2023A left ventricle pseudoaneurysm (LVPA) occurs when the left ventricle free wall rupture becomes contained by pericardium or adhesions. It is rare and has a poor...
UNLABELLED
A left ventricle pseudoaneurysm (LVPA) occurs when the left ventricle free wall rupture becomes contained by pericardium or adhesions. It is rare and has a poor prognosis. LVPA is strongly associated with myocardial infarction. Surgical management of LVPA carries a high mortality rate but is still recommended for most cases of LVPA as soon as the diagnosis is confirmed. Medical management is generally limited to asymptomatic, incidentally found lesions. We present a case of LVPA without any usual risk factors, which was successfully treated by surgery.
LEARNING OBJECTIVES
•To identify the left ventricle pseudoaneurysm (LVPA) that can present with chest pain or dyspnea, but at times can be asymptomatic•To keep a high index of suspicion for LVPA even in patients without the common risk factors such as recent myocardial infarction, cardiac surgery, or trauma•To realize that management options are individualized•To understand that despite a high surgical mortality, for large expanding LVPA, surgery is still recommended•Further research needs to be done to establish management guidelines.
PubMed: 37360827
DOI: 10.1016/j.jccase.2023.03.003 -
JTCVS Techniques Jun 2023Aortic valve repair can be limited by inadequate leaflet tissue for proper coaptation. Various kinds of pericardium have been used for cusp augmentation, but most have...
OBJECTIVES
Aortic valve repair can be limited by inadequate leaflet tissue for proper coaptation. Various kinds of pericardium have been used for cusp augmentation, but most have failed because of tissue degeneration. A more durable leaflet substitute is needed.
METHODS
In this report, 8 consecutive cases are presented in which autologous ascending aortic tissue was used to augment inadequate native cusps during aortic valve repair. Biologically, aortic wall is a living autologous tissue that could have exceptional durability as a leaflet substitute. Techniques for insertion are described in detail, along with procedural videos.
RESULTS
Early surgical outcomes were excellent, with no operative mortalities or complications, and all valves were competent with low valve gradients. Patient follow-up and echocardiograms to a maximum of 8 months' postrepair remain excellent.
CONCLUSIONS
Because of superior biologic characteristics, aortic wall has the potential to provide a better leaflet substitute during aortic valve repair and to expand patient categories amenable to autologous reconstruction. More experience and follow-up should be generated.
PubMed: 37324352
DOI: 10.1016/j.xjtc.2023.02.017 -
Clinical and Experimental Emergency... Dec 2023Myocardial rupture is a fatal complication of acute myocardial infarction (AMI). Early diagnosis of myocardial rupture is feasible when emergency physicians (EPs)...
OBJECTIVE
Myocardial rupture is a fatal complication of acute myocardial infarction (AMI). Early diagnosis of myocardial rupture is feasible when emergency physicians (EPs) perform emergency transthoracic echocardiography (TTE). The purpose of this study was to report the echocardiographic features of myocardial rupture on emergency TTE performed by EPs in the emergency department (ED).
METHODS
This was a retrospective and observational study involving consecutive adult patients presenting with AMI who underwent TTE performed by EPs in the ED of a single academic medical center from March 2008 to December 2019.
RESULTS
Fifteen patients with myocardial rupture, including eight (53.3%) with free wall rupture (FWR), five (33.3%) with ventricular septal rupture (VSR), and two (13.3%) with FWR and VSR, were identified. Fourteen of the 15 patients (93.3%) were diagnosed on TTE performed by EPs. Diagnostic echocardiographic features were found in 100% of the patients with myocardial rupture, including pericardial effusion for FWR and a visible shunt on the interventricular septum for VSR. Additional echocardiographic features indicating myocardial rupture were thinning or aneurysmal dilatation in 10 patients (66.7%), undermined myocardium in six patients (40.0%), abnormal regional motions in six patients (40.0%), and pericardial hematoma in six patients (40.0%).
CONCLUSION
Early diagnosis of myocardial rupture after AMI is possible using echocardiographic features on emergency TTE performed by EPs.
PubMed: 37280049
DOI: 10.15441/ceem.23.037