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Frontiers in Cardiovascular Medicine 2024Severe tricuspid regurgitation (TR) causing cyanosis with patent foramen ovale (PFO) and right-to-left atrial shunting requires a precise diagnosis for optimal therapy....
BACKGROUND
Severe tricuspid regurgitation (TR) causing cyanosis with patent foramen ovale (PFO) and right-to-left atrial shunting requires a precise diagnosis for optimal therapy. Tricuspid valve prolapse (TVP) can lead to TR and is sometimes overlooked, especially in complex cases with factors like pulmonary hypertension (PH). We present an infant with cyanosis and profound TR after high-altitude exposure, initially misattributed to PH but found to be primarily due to spontaneous chordae tendineae rupture and TVP. This case underscores the challenges in diagnosing TR-induced cyanosis.
CASE PRESENTATION
The 3-month-old infant rapidly developed cyanosis, hypoxemia, right atrial enlargement, severe tricuspid regurgitation (TR), and patent foramen ovale (PFO) shunting after high-altitude exposure. Although echocardiography revealed tricuspid valve prolapse (TVP), initial consideration linked TR and right-to-left shunting to pulmonary hypertension (PH) due to the temporal correlation with rapid altitude exposure. Despite hemodynamic stability and the absence of respiratory distress after respiratory support and combined PH medication therapy, the persistent hypoxemia did not reverse as expected. This treatment outcome and repeated echocardiograms reminded us that TR was primarily caused by TVP rather than PH alone. Intraoperative exploration confirmed that TVP was caused by a rupture of TV chordae tendineae and anterior papillary muscle head, and the chordae tendineae/papillary muscle connection was reconstructed. After surgery, this patient was noncyanotic with an excellent long-term prognosis, a trivial TR with normal TV function being observed echocardiographically.
CONCLUSIONS
TR-induced cyanosis can be not only a consequence of PH and right-sided heart dilation but also a primary condition. Repetitive reassessment should be undertaken with caution, particularly when patients are not improving on therapy in the setting of conditions known to predisposition to secondary TR. Since TVP caused by rupture of the chordae or papillary muscles is rare but fatal in children, early diagnosis is clinically substantial to proper management and satisfactory long-term outcomes.
PubMed: 38932987
DOI: 10.3389/fcvm.2024.1335218 -
Diagnostics (Basel, Switzerland) Jun 2024As an integral part of the mitral valve apparatus, the left ventricle papillary muscle (PM) controls mitral valve closure during systole and participates in the ejection... (Review)
Review
As an integral part of the mitral valve apparatus, the left ventricle papillary muscle (PM) controls mitral valve closure during systole and participates in the ejection process during left ventricular systole. Mitral regurgitation (MR) is the most immediate and predominant result when the PM is structurally or functionally abnormal. However, dysfunction of the PM is easily underestimated or overlooked in clinical interventions for MR-related diseases. Therefore, adequate recognition of PM dysfunction and PM-derived MR is critical. In this review, we systematically describe the normal anatomical variations in the PM and the pathophysiology of PM dysfunction-related diseases and summarize the commonly used parameters and the advantages and disadvantages of various noninvasive imaging modalities for the structural and functional assessment of the PM.
PubMed: 38928685
DOI: 10.3390/diagnostics14121270 -
SAGE Open Medical Case Reports 2024Midventricular obstruction (MVO) is a rare form of hypertrophic cardiomyopathy (HCM). While surgical treatment for HCM is among the most technically challenging cardiac...
Midventricular obstruction (MVO) is a rare form of hypertrophic cardiomyopathy (HCM). While surgical treatment for HCM is among the most technically challenging cardiac operations for acquired disease, surgery for MVO is rarely reported. A 38-year-old man was admitted to our hospital with a cough and dyspnea. Transthoracic and transesophageal echography and computed tomography revealed extensive left ventricular hypertrophy, extending from the anteroseptal wall to the apex, and marked papillary muscle hypertrophy. We underwent septal myectomy via aortotomy (Morrow procedure) and apical surgery. Extended myectomy provides the best exposure to the hypertrophied septum and improves the functional status of patients.
PubMed: 38903183
DOI: 10.1177/2050313X241263704 -
Head and Neck Pathology Jun 2024The aim of the study is to investigate the immunohistochemical expression of both Alpha smooth muscle actin and Transforming Growth Factor beta and compare their... (Comparative Study)
Comparative Study
PURPOSE
The aim of the study is to investigate the immunohistochemical expression of both Alpha smooth muscle actin and Transforming Growth Factor beta and compare their expression in oral papillary squamous cell carcinoma with their expression in different histological grades of oral squamous cell carcinoma. A correlation between these immuno-histochemical expressions and histological findings will then be performed. The research question is "Do the percentages of α-SMA and TGF-β immune-expression in OPSCC differ from that in the conventional OSCC?".
METHODS
This will be achieved by collecting archival blocks of oral papillary squamous cell carcinoma and different grades of oral squamous cell carcinoma, staining the specimens with Transforming Growth Factor beta and alpha smooth muscle actin, then measuring the mean staining index of expression in each group and the area percent of both markers.
RESULTS
Results revealed that transforming growth factor beta expression in the epithelium was high in all cases of well-differentiated squamous cell carcinoma, most oral papillary squamous cell carcinoma, and poorly differentiated oral squamous cell carcinoma. On the other hand, different grades of oral squamous cell carcinoma showed a high staining index of alpha smooth muscle actin expression in the stroma. While cases of oral papillary squamous cell carcinoma were either moderate or low-staining.
CONCLUSIONS
Oral papillary squamous cell carcinoma has a favourable prognosis compared to different histological grades, and the prognosis does not depend only on histological grade but also on other prognostic factors.
Topics: Humans; Mouth Neoplasms; Immunohistochemistry; Actins; Biomarkers, Tumor; Carcinoma, Squamous Cell; Squamous Cell Carcinoma of Head and Neck; Transforming Growth Factor beta; Male; Female
PubMed: 38884825
DOI: 10.1007/s12105-024-01635-4 -
JACC. Clinical Electrophysiology May 2024Comparative efficacy and safety data on radiofrequency ablation (RFA) versus pulsed field ablation (PFA) for common idiopathic left ventricular arrhythmia (LV-VAs)...
BACKGROUND
Comparative efficacy and safety data on radiofrequency ablation (RFA) versus pulsed field ablation (PFA) for common idiopathic left ventricular arrhythmia (LV-VAs) locations are lacking.
OBJECTIVES
This study sough to compare RFA with PFA of common idiopathic LV-VAs locations.
METHODS
Ten swine were randomized to PFA or RFA of LV interventricular septum, papillary muscle, LV summit via distal coronary sinus, and LV epicardium via subxiphoid approach. Ablations were delivered using an investigational dual-energy (RFA/PFA) contact force (CF) and local impedance-sensing catheter. After 1-week survival, animals were euthanized for lesion assessment.
RESULTS
A total of 55 PFA (4 applications/site of 2.0 KV, target CF ≥10 g) and 36 RFA (CF ≥10 g, 25-50 W targeting ≥50 Ω local impedance drop, 60-second duration) were performed. LV interventricular septum: average PFA depth 7.8 mm vs RFA 7.9 mm (P = 0.78) and no adverse events. Papillary muscle: average PFA depth 8.1 mm vs RFA 4.5 mm (P < 0.01). Left ventricular summit: average PFA depth 5.6 mm vs RFA 2.7 mm (P < 0.01). Steam-pop and/or ventricular fibrillation in 4 of 12 RFA vs 0 of 12 PFA (P < 0.01), no ST-segment changes observed. Epicardium: average PFA depth 6.4 mm vs RFA 3.3 mm (P < 0.01). Transient ST-segment elevations/depressions occurred in 4 of 5 swine in the PFA arm vs 0 of 5 in the RFA arm (P < 0.01). Angiography acutely and at 7 days showed normal coronaries in all cases.
CONCLUSIONS
In this swine study, compared with RFA, PFA of common idiopathic LV-VAs locations produced deeper lesions with fewer steam pops. However, PFA was associated with higher rates of transient ST-segment elevations and depressions with direct epicardium ablation.
PubMed: 38878017
DOI: 10.1016/j.jacep.2024.04.025 -
Frontiers in Oncology 2024it aimed to explore the value of multislice helical computed tomography (MSCT) in the diagnosis and surgical treatment of primary tracheal tumors.
OBJECTIVE
it aimed to explore the value of multislice helical computed tomography (MSCT) in the diagnosis and surgical treatment of primary tracheal tumors.
METHODS
64 patients with the primary tracheal tumor who were diagnosed in Wuxi Second People's Hospital from March 2020 to March 2021 were selected as the research objects. MSCT imaging was performed on all patients, and suitable surgical methods. The pathological results were compared with original CT, CT virtual endoscopy (CTVE), and Comparisons were made using CT three-dimensional reconstruction images to evaluate the accuracy of MSCT diagnosis. Parameters such as postoperative complications and survival rates were observed to assess surgical effectiveness and safety.
RESULTS
Compared with original CT images (70%, 72%, 70%), the diagnostic accuracy of VR images (80%, 80%, 80%), MPVR images (85%, 90%, 92%), and CTVE images (100%, 100%, 100%) was remarkably improved (P<0.05). The three-year survival rate of patients with smooth muscle tumors, malignant tumors, salivary gland adenoma, papillary tumors, and inflammatory polyp was markedly lower than that of the one-year survival rate, with a significant difference (P<0.05). The incidence of postoperative complications was 14.1%, with three cases resulting in complication-related deaths.
CONCLUSION
the diagnostic accuracy of MSCT imaging of primary tracheal tumor was high. The diagnostic accuracy of CTVE was higher than that of VR and MPVR. Besides, surgical treatment of primary tracheal tumor had a substantial effect, with no serious postoperative complications.
PubMed: 38860002
DOI: 10.3389/fonc.2024.1376228 -
A rare case of retroperitoneal teratoma with evidence of papillary thyroid carcinoma: a case report.BMC Endocrine Disorders Jun 2024Teratomas are germ cell tumors composed of somatic tissues from up to three germ layers. Primary retroperitoneal teratomas usually develop during childhood and are...
BACKGROUND
Teratomas are germ cell tumors composed of somatic tissues from up to three germ layers. Primary retroperitoneal teratomas usually develop during childhood and are uncommon in adults and in the retroperitoneal space. While there are only a few cases of retroperitoneal thyroid tissue, we report a unique case of a retroperitoneal papillary thyroid carcinoma.
CASE PRESENTATION
A 41-year-old woman presented in our institution due to intermitted unspecific abdominal pain. Magnetic resonance imaging detected a multi-cystic solid retroperitoneal mass ventral to the psoas muscle and the left iliac artery. After surgical removal of the retroperitoneal mass, histology sections of the specimen indicated evidence of papillary thyroid carcinoma cells. A staging computed tomography scan of the body showed no further manifestations. To reduce the risk of recurrence, total thyroidectomy was performed followed by radioiodine therapy with lifelong hormone substitution.
CONCLUSIONS
Primary retroperitoneal teratoma with evidence of papillary thyroid carcinoma is a rare condition. Preoperative diagnosis is difficult due to its non-specific clinical manifestation and lack of specific radiologic findings. Histopathology analysis is necessary for diagnosis. Although surgery is considered the first line treatment, there is still discussion about the extent of resection and the need for total thyroidectomy with adjuvant radioiodine therapy.
Topics: Humans; Female; Adult; Teratoma; Retroperitoneal Neoplasms; Thyroid Neoplasms; Thyroid Cancer, Papillary; Thyroidectomy; Prognosis
PubMed: 38858658
DOI: 10.1186/s12902-024-01606-4 -
European Journal of Case Reports in... 2024Alagille syndrome (ALGS) is a multisystem disorder involving at least three systems among the liver, heart, skeleton, face, and eyes. Common cardiac associations include...
BACKGROUND
Alagille syndrome (ALGS) is a multisystem disorder involving at least three systems among the liver, heart, skeleton, face, and eyes. Common cardiac associations include pulmonary artery stenosis/atresia, atrial septal defect (ASD), ventricular septal defect (VSD) and tetralogy of fallot (ToF). Coarctation of aorta (CoA), renal and intracranial arteries are commonly involved vessels in Alagille syndrome. We present two cases with rare cardiovascular manifestations of Alagille syndrome. .
CASE 1
A 25-year-old female with a history of Alagille syndrome presented to the cardiologist office for progressive exertional dyspnoea, orthopnoea, and palpitations. She was tachycardiac on examination and had an apical diastolic rumble. A transthoracic echocardiogram (TTE) showed a left ventricular ejection fraction (LVEF) of 60% and parachute mitral valve (PMV) with severe mitral stenosis. A transoesophageal echocardiogram (TOE) showed insertion of chordae into the anterolateral papillary muscle, severe mitral stenosis with a valve area of 0.7 cm. She was referred to a congenital heart disease specialist and underwent robotic mitral valve replacement with improvement in her symptoms.
CASE 2
A 27-year-old female with known Alagille syndrome and resistant hypertension presented to the cardiologist office due to progressive exertional dyspnoea for a year. She was hypertensive and had a new 2/6 systolic ejection murmur along the left upper sternal border. TTE revealed an LVEF of 60% and pulmonary artery pressure of 19 mmHg. A CoA was suspected distal to the left subclavian artery due to a peak gradient of 38 mmHg. Cardiac magnetic resonance (CMR) imaging ruled out CoA, and diffuse narrowing of the descending thoracic aorta measuring 13-14 mm in diameter was noted. The patient was referred to a congenital heart disease specialist for further management.
CONCLUSION
PMV presenting as mitral stenosis and mid-aortic syndrome are not commonly described anomalies in association with Alagille syndrome. TTE, TOE and CMR played a key role in diagnosis and management of these patients.
LEARNING POINTS
Alagille syndrome (ALGS) is a complex multisystem disorder involving the liver, heart, skeleton, face, and eyes. Cardiovascular involvement occurs in up to 95% of the patients.Common cardiac associations include pulmonary artery stenosis/atresia, atrial septal defect (ASD), ventricular septal defect (VSD) and tetralogy of fallot (ToF).A parachute mitral valve (PMV) presenting as mitral stenosis and mid-aortic syndrome is not commonly described anomalies in association with ALGS. Here, we present such rare cases.
PubMed: 38846669
DOI: 10.12890/2024_004545 -
Cureus May 2024Urinary bladder cancer (UBC) is amongst the most common urological malignancies.
BACKGROUND
Urinary bladder cancer (UBC) is amongst the most common urological malignancies.
AIM
To study different types of urinary bladder lesions in the north Indian population and to correlate various clinical and pathological findings.
MATERIALS AND METHODS
The present prospective study was conducted on 100 cases undergoing transurethral resection of bladder tumor (TURBT) and/or radical cystectomy over a period of 2.5 years followed by histopathological examination. Liquid-based cytology for malignant cells in urine was also performed. Immunohistochemistry was employed for tumor typing wherever needed.
RESULTS
A total of 100 cases were studied. Male to female ratio was 15.7:1 and most of the patients were in the sixth decade (40%). Painless hematuria was the commonest clinical presentation (60%) and smoking was the commonest risk factor (80%). The most common lesion was infiltrating urothelial carcinoma seen in 72 cases followed by papillary urothelial neoplasm of low malignant potential (PUNLMP) seen in eight cases. Grade and depth of invasion were assessed and correlated. Several variants of infiltrating urothelial carcinoma such as squamous differentiation, glandular differentiation, microcystic, clear cell, nested, and micropapillary were also identified. Clinical, cystoscopic and histopathological findings were correlated in all the cases.
CONCLUSION
Infiltrating urothelial carcinoma high grade was the most common bladder lesion identified and muscle invasion was more common with higher-grade lesions. A decade-younger age group was found to be more affected in the present series. Urine cytology for malignant cells is useful for early diagnosis of cancer. Immunohistochemistry is an important ancillary adjunct.
PubMed: 38846211
DOI: 10.7759/cureus.59792