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International Journal of Surgery Case... Nov 2023The sternum, pericardium, diaphragm, abdominal wall, and heart are all affected by the unusual congenital condition known as Cantrell pentalogy. It is a rare congenital...
INTRODUCTION
The sternum, pericardium, diaphragm, abdominal wall, and heart are all affected by the unusual congenital condition known as Cantrell pentalogy. It is a rare congenital disorder that requires multidisciplinary care. Early diagnosis and appropriate management are crucial for improving outcomes in affected individuals. To illustrate the difficulties and complexity of Cantrell pentalogy, we provide two cases.
PRESENTATION OF CASE
In case 1, a routine antenatal scan at 12 weeks' gestation revealed thoracoabdominal ectopia cordis in a 29-year-old woman. The pregnancy was terminated medically due to the severity of the anomalies and the poor prognosis. A 32-year-old patient in case 2 had a diaphragmatic hernia, thoracoabdominal ectopia cordis and midline abdominal wall abnormalities. After counselling, a medical termination was chosen. The ultra-sonographic features were confirmed by autopsy results in 2 cases.
DISCUSSION
Early diagnosis is feasible in the first trimester if ectopia cordis and omphalocele exist. Additionally, development in ultrasound technology provides us with better visualization and early diagnosis. With patients who have fully developed Cantrell syndrome and those who also have accompanying anomalies, the prognosis is often poor, with short survival and quality of life.
CONCLUSIONS
Cantrell pentalogy is a rather uncommon congenital condition. Early detection is possible in the first trimester. The severity of the illness varies greatly, and treatment is determined by the precise abnormalities present. Early diagnosis necessitates adequate initial training as well as ongoing in-service training for sonographers. Early detection and treatment are critical for improving outcomes in affected persons.
PubMed: 37879290
DOI: 10.1016/j.ijscr.2023.108941 -
Heart Rhythm Jun 2024Bipolar radiofrequency ablation (B-RFA) is a method used to treat the arrhythmia substrate resistant to unipolar ablation. Few studies have addressed endo-epicardial...
BACKGROUND
Bipolar radiofrequency ablation (B-RFA) is a method used to treat the arrhythmia substrate resistant to unipolar ablation. Few studies have addressed endo-epicardial B-RFA.
OBJECTIVE
The aim of the study was to evaluate chronic lesions resulting from endo-epicardial B-RFA and to determine optimal settings for such procedures in an animal model.
METHODS
In 7 pigs, up to 5 radiofrequency applications per animal were performed with 2 electrodes placed on both sides of the left ventricular free wall. Current was delivered for 60 seconds by a generator dedicated for B-RFA with power settings of 25, 30, 35, 40, and 50 W.
RESULTS
At 12 weeks after ablation, 31 lesions were assessed. Their maximal cross-sectional area ranged from 7.2 to 68 mm and correlated with total power delivered (r = 0.53), with temperature increment at the endocardial catheter (r = 0.65), and inversely with temperature decrement at the epicardial catheter (r = 0.54). For power values between 30 and 40 W, the lesion area did not differ significantly (P = .92). Lesion depth ranged from 1.9 to 11 mm and correlated with impedance decrement (r = 0.5). Lesions were transmural in 8 cases. Lesion depth/wall thickness ratio was on average 0.6 ± 0.3, with the smallest value for 25 W (0.5 ± 0.3) and the largest for 50 W (0.8 ± 0.3). Steam pops occurred at a power range of 30-50 W, with an incidence of 1 in 5 applications, with 1 case of fatal tamponade at 40 W. Impedance decrement, endocardial catheter temperature increment, and endocardial electrogram amplitude decrement were greater during applications with steam pops.
CONCLUSION
Chronic lesions resulting from endo-epicardial B-RFA appear smaller and less often transmural compared with acute lesions described in the literature. The incidence of steam pops during endo-epicardial B-RFA is relatively high even at low powers.
Topics: Animals; Feasibility Studies; Swine; Pericardium; Endocardium; Disease Models, Animal; Catheter Ablation; Heart Ventricles; Equipment Design
PubMed: 38336196
DOI: 10.1016/j.hrthm.2024.02.009 -
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 -
Ochsner Journal 2023Most pericardial effusions that occur in the setting of ST-segment elevation myocardial infarction (STEMI) are small, simple, and without symptomology. However, in its...
Most pericardial effusions that occur in the setting of ST-segment elevation myocardial infarction (STEMI) are small, simple, and without symptomology. However, in its most severe form, pericardial effusion can precipitate cardiac tamponade, and when untreated, can cause abrupt hemodynamic instability. Pericardial effusion may be a manifestation of left ventricular free-wall rupture, hemorrhagic pericarditis, or aortic dissection involving a coronary artery. We describe the case of a 65-year-old male who experienced chest pain for several days prior to admission but delayed seeking care because he wished to avoid coronavirus disease 2019 exposure. Upon arrival, he was hemodynamically unstable. Electrocardiogram was consistent with anterior STEMI. Bedside echocardiogram demonstrated a hypertrophic left ventricle with preserved function and a large, complex pericardial effusion with cardiac tamponade physiology. Computed tomography of the chest identified hemopericardium but was unable to delineate etiology. The patient underwent emergent thoracotomy because of persistent shock, and during the surgery, left ventricular free-wall rupture was identified and repaired. Coronary artery bypass grafting to the patient's left anterior descending artery was also performed. The patient remained asymptomatic at 2-year follow-up. The differential for hemodynamic compromise in a patient with STEMI is broad, but quickly distinguishing pump failure from other life-threatening causes of shock is imperative to dictate time-sensitive management decisions. The presence of a hemorrhagic pericardial effusion in the setting of STEMI is a surrogate marker for a severe infarct and can help the bedside physician determine whether a patient will be better served in the catheterization lab for revascularization or in the operating room for surgical repair.
PubMed: 37711482
DOI: 10.31486/toj.23.0023 -
European Heart Journal. Case Reports May 2024Secondary cardiac tumours are much more common compared with primary (100-1000 times). The majority of the primary cardiac tumours are benign; however, almost a quarter...
BACKGROUND
Secondary cardiac tumours are much more common compared with primary (100-1000 times). The majority of the primary cardiac tumours are benign; however, almost a quarter are malignant, and 95% of these are sarcomas. The rarest type of primary malignant cardiac sarcoma is intimal (spindle cell) sarcoma.
CASE SUMMARY
A 37-year-old woman presented with episodes of breathlessness. Initially treated for a chest infection, however, the patient continued to deteriorate and presented to the emergency department. A large pericardial effusion was discovered and drained, with samples sent for analysis. A repeat interval echo confirmed the resolution of the pericardial effusion with preserved left ventricular (LV) systolic function. The computed tomography (CT) of the thorax showed suspicious lesions in the heart and lung while the repeat echo raised suspicion of an infiltrative disease. A cardiac magnetic resonance imaging scan was performed, which suggested evidence of an undifferentiated sarcoma involving the posterior wall of the LV and an overlying thrombus. Computed tomography of the abdomen and pelvis did not show any evidence of abdominal metastasis. A CT-guided lung biopsy was arranged. On histological analysis, the report was overall strongly supportive of a diagnosis of intimal sarcoma. She underwent chemotherapy until recently.
DISCUSSION
Cardiac intimal sarcomas are the least reported type of primary malignant tumours of the heart. They are encountered more commonly in the large arterial blood vessels, including the pulmonary artery and aorta, and are extremely rare in the heart. A prompt diagnosis is essential as they are considered extremely aggressive.
PubMed: 38770404
DOI: 10.1093/ehjcr/ytae072 -
Heart Failure Clinics Jul 2024Cardiac magnetic resonance represents the gold standard imaging technique to assess cardiac volumes, wall thickness, mass, and systolic function but also to provide... (Review)
Review
Cardiac magnetic resonance represents the gold standard imaging technique to assess cardiac volumes, wall thickness, mass, and systolic function but also to provide noninvasive myocardial tissue characterization across almost all cardiac diseases. In patients with cardiac amyloidosis, increased wall thickness of all heart chambers, a mildly reduced ejection fraction and occasionally pleural and pericardial effusion are the characteristic morphologic anomalies. The typical pattern after contrast injection is represented by diffuse areas of late gadolinium enhancement, which can be focal and patchy in very early stages, circumferential, and subendocardial in intermediate stages or even diffuse transmural in more advanced stages.
Topics: Humans; Amyloidosis; Cardiomyopathies; Magnetic Resonance Imaging, Cine; Contrast Media; Magnetic Resonance Imaging; Myocardium; Stroke Volume
PubMed: 38844300
DOI: 10.1016/j.hfc.2024.03.002 -
Academic Radiology Nov 2023The aim of this study was to assess the prevalence of reportable cardiac findings detected on abdominopelvic CTs and the association with subsequent cardiovascular...
PURPOSE
The aim of this study was to assess the prevalence of reportable cardiac findings detected on abdominopelvic CTs and the association with subsequent cardiovascular events.
MATERIALS AND METHODS
We performed a retrospective search of electronic medical record of patients who underwent abdominopelvic CT between November 2006 and November 2011 with a clinical history of upper abdominal pain. A radiologist blinded to the original CT report reviewed all 222 cases for the presence of pertinent reportable cardiac findings. The original CT report was also evaluated for documentation of pertinent reportable cardiac findings. The following findings were recorded on all CTs: presence of coronary calcification, fatty metaplasia, ventricle wall thinning and thickening, valve calcification or prosthesis, heart/chamber enlargement, aneurysm, mass, thrombus, device, air within ventricles, abnormal pericardium, prior sternotomy, and adhesions if prior sternotomy. Medical records were reviewed to identify cardiovascular events on follow-up in patients with the presence or absence of cardiac findings. We compared the distribution findings in patients with and without cardiac events using the Wilcoxon test (for continuous variables) and the Pearson's chi-squared test (for categorical variables).
RESULTS
Eighty-five of 222 (38.3%) patients (52.7% females, median age 52.5 years) had at least one pertinent reportable cardiac finding on the abdominopelvic CT, with a total of 140 findings in this group. From the total 140 findings, 100 (71.4%) were not reported. The most common findings seen on abdominal CTs were: coronary artery calcification (66 patients), heart or chamber enlargement (25), valve abnormality (19), sternotomy and surgery signs (9), LV wall thickening (7), device (5), LV wall thinning (2), pericardial effusion (5), and others (3). After a mean follow-up of 43.9 months, 19 cardiovascular events were found in the cohort (transient ischemic attack, cerebrovascular accident, myocardial infarction, cardiac arrest, acute arrhythmia, palpitation, syncope and acute chest pain). Only 1 event occurred in the group of patients with no incidental pertinent reportable cardiac findings (1/137 = 0.73%). All other 18 events occurred in patients with incidental pertinent reportable cardiac findings (18/85 = 21.2%), which was significantly different (p < 0.0001). One out of the total 19 events in the overall group (5.24%) occurred in a patient with no incidental pertinent reportable cardiac findings while 18 of 19 total events (94.74%) occurred with patients with incidental pertinent reportable cardiac findings, which was also significantly different (p < 0.001). Fifteen of the total events (79%) occurred in patients in whom the incidental pertinent reportable cardiac findings were not reported, which was significantly different (p < 0.001) from the four events that occurred in patients in whom the incidental pertinent reportable cardiac findings were reported or had no findings.
CONCLUSIONS
Incidental pertinent reportable cardiac findings are common on abdominal CTs and are frequently not reported by radiologists. These findings are of clinical relevance since patients with pertinent reportable cardiac findings have a significantly higher incidence of cardiovascular events on follow-up.
PubMed: 36872179
DOI: 10.1016/j.acra.2023.01.026 -
Journal of Cardiovascular... Sep 2023Epicardial ablation is an important approach in the management of patients with complex ventricular arrhythmias. Irrigated ablation catheters present a challenge in this...
INTRODUCTION
Epicardial ablation is an important approach in the management of patients with complex ventricular arrhythmias. Irrigated ablation catheters present a challenge in this potential space due to fluid accumulation that can cause hemodynamic compromise, requiring frequent manual fluid aspiration. In this series, we report our initial experience with the use of a dry suction water seal system for pericardial fluid management during epicardial ablation.
METHODS
Consecutive patients undergoing epicardial ventricular tachycardia (VT) ablation at a single center were included. All patients underwent epicardial access via a subxiphoid approach with a single operator. A deflectable sheath was advanced into the pericardial space, and the side port was attached to a dry suction water seal system attached to wall suction at -20 mmHg. Procedural information including patient characteristics, outcomes, and adverse events. After a period of initial experience, pericardial fluid infusion and aspiration volumes were recorded.
RESULTS
Eleven patients were included in this series. All patients underwent epicardial ablation with complete success achieved in 8 of the 11 patients and partial success in the remaining patients. Pericardial fluid intake ranging from 485 to 3050 mL with aspiration of 350-3050 mL using the dry suction water seal system. No adverse events occurred.
CONCLUSION
Dry suction water seal drainage systems can provide a safe strategy for efficient pericardial fluid management during epicardial VT ablation, potentially shortening procedure duration.
Topics: Humans; Pericardial Fluid; Tachycardia, Ventricular; Suction; Pericardium; Catheter Ablation; Epicardial Mapping
PubMed: 37529856
DOI: 10.1111/jce.16017 -
International Journal of Surgery Case... Dec 2023Mediastinal teratoma is the most common mediastinal germ cell tumor. Mature mediastinal teratomas are infrequent and often found incidentally.
INTRODUCTION AND IMPORTANCE
Mediastinal teratoma is the most common mediastinal germ cell tumor. Mature mediastinal teratomas are infrequent and often found incidentally.
CASE PRESENTATION
We report the case of a 14-year-old girl who was presented dyspnea for 2 years. A computed tomography of the chest revealed a 19 cm heterogenous right-sided anterior mediastinal mass suggesting mature teratoma. The mass compressed vital structures. The tumor was considered resectable. By hemiclamshell approach, we punctured the tumor and aspirated its contents. The tumor was totally resected en bloc with a cuff of pericardium, phrenic nerve and azygos vena. The pericardial defect was repaired using Mersilene mesh. Histopathology of the tumor revealed a mature cystic teratoma. Postoperative course was uneventful.
CLINICAL DISCUSSION
Complete surgical excision is the treatment of choice for mediastinal mature teratoma. The choice of incision for removing the tumor depend on the tumor size, location and the relashionships with the associated vital structures. Lengthy incisions were required for the safe mobilization of the tumor. In addition, contents aspiration through a small incision in the giant tumor wall helped improve tumor mobilization.
CONCLUSION
Appropriate surgical strategy for a well selected case maintains functional status and results total tumor resection.
PubMed: 37952494
DOI: 10.1016/j.ijscr.2023.109003 -
Immunity, Inflammation and Disease Mar 2024Systemic lupus erythematosus (SLE) is a multisystem-involved, highly heterogeneous autoimmune disease with diverse clinical manifestations. We report an extremely rare...
OBJECTIVE
Systemic lupus erythematosus (SLE) is a multisystem-involved, highly heterogeneous autoimmune disease with diverse clinical manifestations. We report an extremely rare case of SLE with severe diffuse myocardial hypertrophy.
METHODS
The patient's echocardiography and cardiac magnetic resonance imaging (CMR) results indicated diffuse myocardial hypertrophy. After excluding coronary atherosclerosis, hypertensive cardiomyopathy, drug toxicity, and other causes, the patient was diagnosed with SLE-specific cardiomyopathy. Medications such as hormones, antimalarials, immunosuppressants, and biologics were administered.
RESULTS
Ancillary test results were as follows: hs-cTnI: 0.054 ng/mL (0-0.016); NTproBNP: 1594.0 pg/mL (<150); A contrast-enhanced CMR revealed the diffuse thickening of the left ventricular wall with multiple abnormal enhancements, reduced left ventricular systolic and diastolic function, and moderate amount of pericardial effusion. Endomyocardial myocardial biopsy was performed, showing cardiomyocyte hypertrophy and degeneration, and no changes in myocarditis or amyloidosis. The pathology viewed by electron microscopy showed increased intracellular glycogen in the myocardium, and no hydroxychloroquine-associated damage in the myocardium. The 24-h ambulatory blood pressure and contrast-enhanced computed tomography of coronary arteries were normal. The diagnosis of SLE-specific cardiomyopathy was clear. The myocardial hypertrophy showed reversible alleviation following treatment with high-dose corticosteroids. CMR results before and after treatment were as follows: interventricular septum, pretreatment (28) versus post-treatment (22) mm; left ventricular inferior wall, pretreatment (18-21) versus post-treatment (12-14) mm; left ventricular lateral wall, pretreatment (17-18) versus post-treatment (10-12) mm; pericardial effusion (left ventricular lateral wall), pretreatment (25) versus post-treatment (12) mm; left ventricular ejection fraction, pretreatment (38.9%) versus post-treatment (66%).
CONCLUSION
Myocardial hypertrophy may be an important sign of active and prognostic assessment in SLE diagnosis and management. Similarly, when encountering cases of myocardial hypertrophy, the possibility of autoimmune disease should be considered in addition to common causes.
Topics: Humans; Blood Pressure Monitoring, Ambulatory; Cardiomyopathies; Hypertrophy; Lupus Erythematosus, Systemic; Pericardial Effusion; Stroke Volume; Ventricular Function, Left
PubMed: 38533913
DOI: 10.1002/iid3.1214