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1.
Kyobu Geka ; 77(5): 369-372, 2024 May.
Artículo en Japonés | MEDLINE | ID: mdl-38720607

RESUMEN

An 81-year-old man underwent total arch replacement for thoracic aortic aneurysm 8 years ago and catheter ablation for paroxysmal atrial fibrillation 1 year ago. Transthoracic echocardiography revealed a mass in the right atrium, and the patient was admitted for close examination and treatment. Transesophageal echocardiography revealed a 23×17 mm large well-defined mass above the cavotricuspid isthmus. Two venous drainage cannulas were inserted directly to the superior vena cava and to the inferior vena cava via the right femoral vein, in order to avoid the direct contact with the right atrium prior to institution of cardiopulmonary bypass. The right atrial tumor was found attached to the cavotricuspid isthmus, and was resected together with the right atrial wall. Pathological examination showed myxomatous tissue. Postoperative course was uneventful. He was discharged 23 days after the operation.


Asunto(s)
Ablación por Catéter , Atrios Cardíacos , Neoplasias Cardíacas , Mixoma , Humanos , Mixoma/cirugía , Mixoma/diagnóstico por imagen , Masculino , Anciano de 80 o más Años , Neoplasias Cardíacas/cirugía , Neoplasias Cardíacas/diagnóstico por imagen , Atrios Cardíacos/cirugía
2.
Zhonghua Yi Xue Za Zhi ; 104(18): 1628-1630, 2024 May 14.
Artículo en Chino | MEDLINE | ID: mdl-38742351

RESUMEN

A total of 25 patients with right cardiac system tumors in the Department of Cardiac Surgery, Beijing Anzhen Hospital from January 2012 to October 2022 were retrospectively included in the study. The preoperative data, and information of surgical treatment and perioperative management on these patients were analyzed and summarized. One patient developed pulmonary embolism and died before surgery, and the other 24 patients (16 males and 8 females) received surgical treatment, with an average age of (44.7±10.2) years (24-74 years). Nine patients were diagnosed with malignant tumors. Among the 24 patients who received surgical treatment, two patients died during the perioperative period, in-situ tumor recurrence was seen in three patients within about 1 year after surgery (two patients died without surgery, and one patient died 3 months after surgery), two patients had distant metastasis, and 17 patients had a good prognosis. Right cardiac system tumors are rare, with a high malignant rate, and the clinical manifestations vary greatly. Active surgical intervention is found to be effective, and the prognosis is closely related to the pathological type and extent of tumor invasion.


Asunto(s)
Neoplasias Cardíacas , Humanos , Persona de Mediana Edad , Masculino , Adulto , Femenino , Neoplasias Cardíacas/cirugía , Estudios Retrospectivos , Anciano , Pronóstico , Recurrencia Local de Neoplasia , Adulto Joven
3.
J Cardiothorac Surg ; 19(1): 168, 2024 Apr 02.
Artículo en Inglés | MEDLINE | ID: mdl-38566159

RESUMEN

Cardiac leiomyosarcomas are a rare subset of the already infrequent, primary malignant cardiac neoplasia spectrum. The most common site for a primary leiomyosarcoma of the ventricle is on the right with fewer than five globally reported cases in the left ventricle. Most present with non-specific symptoms but attention is usually sought after the appearance of compressive symptoms or arrhythmias. We present a case of a left ventricular leiomyosarcoma in a 50-year old female patient that had a delayed diagnosis and its subsequent surgical resection and oncological management with docetaxel and gemcitabine. This case highlights the need for a high index of suspicion for cardiac masses especially if there are competing chronic diseases with similar symptomatology. Given the rare presentation of left ventricular leiomyosarcomas, case reports may provide valuable information that is otherwise unavailable.


Asunto(s)
Neoplasias Cardíacas , Leiomiosarcoma , Femenino , Humanos , Persona de Mediana Edad , Ventrículos Cardíacos/patología , Leiomiosarcoma/diagnóstico , Leiomiosarcoma/cirugía , Leiomiosarcoma/patología , Neoplasias Cardíacas/diagnóstico , Neoplasias Cardíacas/cirugía , Neoplasias Cardíacas/patología
5.
Zhonghua Bing Li Xue Za Zhi ; 53(4): 358-363, 2024 Apr 08.
Artículo en Chino | MEDLINE | ID: mdl-38556819

RESUMEN

Objective: To assess the clinicopathological features, immunophenotype, molecular characteristics and differential diagnosis of primary cardiac synovial sarcoma (PCSS). Methods: Five cases of PCSS were collected at Guangdong Provincial People's Hospital from 2008 to 2023, and their clinicopathological features were summarized. Immunohistochemical staining, fluorescence in-situ hybridization (FISH) and next-generation sequencing (NGS) were performed, and relevant literatures were reviewed. Results: The cases were found in four males and one female, ranging in ages from 16 to 51 years (median 30 years). Two cases were located in the pericardium, two in the right ventricle, and one in the left ventricle. Follow-up data were available in four cases. All the four patients died of disease at 3, 7, 13 and 26 months, respectively, after diagnosis. The tumor maximum diameter ranged from 6.0 to 14.0 cm in (mean 10.0 cm). Microscopically, three cases were monophasic and two cases were biphasic. Immunohistochemically, all cases were immunoreactive for EMA, vimentin, bcl-2 and CD56. The tumor cells were variably positive for pan-cytokeratin, SS18-SSX, SOX2, TLE1, CD99, synaptophysin, calretinin and calponin. FISH showed the presence of SS18 rearrangement in all the cases. NGS detected SS18-SSX gene fusion in three cases (SS18-SSX1 in one and SS18-SSX2 in two). Conclusions: PCSS is an exceedingly rare neoplasm, and should be distinguished from other various malignant epithelial and mesenchymal tumors. The clinical history, histopathological and immunohistochemical features, and molecular findings are all essential to the definitive diagnosis of PCSS.


Asunto(s)
Neoplasias Cardíacas , Neoplasias del Mediastino , Sarcoma Sinovial , Masculino , Humanos , Femenino , Biomarcadores de Tumor/genética , Biomarcadores de Tumor/análisis , Sarcoma Sinovial/genética , Sarcoma Sinovial/diagnóstico , Sarcoma Sinovial/patología , Proteínas Proto-Oncogénicas/genética , Proteínas Represoras/genética , Proteínas de Fusión Oncogénica/genética , Neoplasias Cardíacas/genética , Neoplasias Cardíacas/cirugía
6.
Kyobu Geka ; 77(4): 311-314, 2024 Apr.
Artículo en Japonés | MEDLINE | ID: mdl-38644180

RESUMEN

The patient is a 76-year-old man. His chief complaint of chest pain led to a diagnosis of pericardial effusion of unknown cause, and pericardial drainage was performed. On the 30th day, chest pain appeared again. Echocardiography revealed a pericardial fluid reaccumulation and a substantial mass in the pericardial space. Surgical drainage was performed to find the cause. A hematoma/mass was present on the epicardium. The pericardial sac was filled with hematoma. The hematoma was removed, but part of the mass infiltrated close to the anterior descending branch of the left coronary artery, and removal of that part was abandoned. The intrapericardial hematoma and epicardium were submitted to pathology leading to the diagnosis of synovial sarcoma. The patient was discharged home 14 days after surgery.


Asunto(s)
Neoplasias Cardíacas , Derrame Pericárdico , Sarcoma Sinovial , Humanos , Masculino , Sarcoma Sinovial/complicaciones , Sarcoma Sinovial/cirugía , Sarcoma Sinovial/diagnóstico por imagen , Derrame Pericárdico/etiología , Derrame Pericárdico/diagnóstico por imagen , Derrame Pericárdico/cirugía , Anciano , Neoplasias Cardíacas/complicaciones , Neoplasias Cardíacas/cirugía , Neoplasias Cardíacas/diagnóstico por imagen , Recurrencia
7.
Kyobu Geka ; 77(4): 316-318, 2024 Apr.
Artículo en Japonés | MEDLINE | ID: mdl-38644181

RESUMEN

Calcified amorphous tumor (CAT), a non-neoplastic tumor, is rare. Histopathologic features are the presence of calcified nodules in an amorphous background of fibrin. CAT is reported to be associated with renal dysfunction or hemodialysis, and possibly causes cerebral embolism. We report a case of CAT diagnosed after stroke. A 58-year-old male with a 2-year history of hemodialysis was diagnosed with an acute stroke, and was treated medically. Paralysis promptly improved, but transthoracic echocardiography revealed a tumor attached to the posterior mitral leaflet and dense mitral annular calcification. To prevent embolism due to the large tumor, we performed resection of the tumor. Pathological findings showed calcifications surrounded by amorphous fibrous tissue, indicating CAT. Postoperative course was uneventful.


Asunto(s)
Calcinosis , Accidente Cerebrovascular , Humanos , Masculino , Persona de Mediana Edad , Calcinosis/diagnóstico por imagen , Calcinosis/cirugía , Accidente Cerebrovascular/etiología , Accidente Cerebrovascular/diagnóstico por imagen , Neoplasias Cardíacas/cirugía , Neoplasias Cardíacas/diagnóstico por imagen , Neoplasias Cardíacas/complicaciones
8.
J Cardiothorac Surg ; 19(1): 229, 2024 Apr 16.
Artículo en Inglés | MEDLINE | ID: mdl-38627746

RESUMEN

An 80-year-old female was referred to our institution due to transient right upper limb weakness. Transthoracic and transesophageal echocardiography revealed a tumor in the left atrium. The tumor was attached to the posterior wall of the left atrium near the atrioventricular node. Intraoperative pathological examination revealed that the tumor was a myxoma, and complete resection was successfully performed. However, she experienced persistent complete atrioventricular block postoperatively and required pacemaker implantation.


Asunto(s)
Bloqueo Atrioventricular , Neoplasias Cardíacas , Mixoma , Femenino , Humanos , Anciano de 80 o más Años , Ecocardiografía , Bloqueo Atrioventricular/etiología , Bloqueo Atrioventricular/cirugía , Neoplasias Cardíacas/complicaciones , Neoplasias Cardíacas/diagnóstico por imagen , Neoplasias Cardíacas/cirugía , Atrios Cardíacos/diagnóstico por imagen , Mixoma/complicaciones , Mixoma/diagnóstico por imagen , Mixoma/cirugía
9.
BMC Anesthesiol ; 24(1): 159, 2024 Apr 25.
Artículo en Inglés | MEDLINE | ID: mdl-38664657

RESUMEN

Serratus posterior intercostal plane block (SPSIPB) is a novel periparavertebral block. It provides anterolateral posterior chest wall analgesia. It is an interfascial plane block, performed under ultrasound guidance, and the visualization of landmarks is easy. It is performed deep into the serratus posterior superior muscle at the level of the third rib. Until now, there have been case reports about the usage of single-shot SPSIPB, but there are no reports about the usage of the block catheterization technique of SPSIPB. Continuous infusion from a catheter of interfascial plane blocks is important for postoperative analgesia management after painful surgeries such as thoracic and cardiac surgeries. Thus, we performed SPSIPB catheterization in a patient who underwent right atrial mass excision with minimally invasive thoracotomy surgery. Here, we present our successful analgesic experience with continuous SPSIPB in this case report.


Asunto(s)
Atrios Cardíacos , Bloqueo Nervioso , Dolor Postoperatorio , Humanos , Bloqueo Nervioso/métodos , Dolor Postoperatorio/tratamiento farmacológico , Dolor Postoperatorio/prevención & control , Atrios Cardíacos/cirugía , Toracotomía/métodos , Ultrasonografía Intervencional/métodos , Masculino , Persona de Mediana Edad , Femenino , Neoplasias Cardíacas/cirugía , Nervios Intercostales
10.
Cancer Rep (Hoboken) ; 7(4): e2033, 2024 Apr.
Artículo en Inglés | MEDLINE | ID: mdl-38600050

RESUMEN

BACKGROUND: Primary cardiac myxofibrosarcoma is a rare and aggressive malignancy, with the majority of approaching strategies relying on case reports. This article provides insights into its diagnosis and treatment. CASE PRESENTATION: This paper presents the case of a 40-year-old man with sudden onset hemoptysis, leading to the diagnosis of primary cardiac myxofibrosarcoma. Treatment involved open-heart surgery to excise the left atrium tumor, followed by 6 cycles of adjuvant chemotherapy. Unfortunately, brain metastasis developed, leading to the patient's death 1 year after initial diagnosis. CONCLUSION: Primary cardiac myxofibrosarcoma remains a clinical challenge with an unfavorable prognosis. Early diagnosis through advanced imaging is crucial, and research is needed to explore innovative treatments. This case underscores the complexities of managing this rare cardiac malignancy and highlights the necessity for ongoing investigations to enhance patient outcomes.


Asunto(s)
Fibrosarcoma , Neoplasias Cardíacas , Neoplasias del Mediastino , Masculino , Adulto , Humanos , Neoplasias Cardíacas/diagnóstico , Neoplasias Cardíacas/cirugía , Neoplasias Cardíacas/patología , Atrios Cardíacos/diagnóstico por imagen , Atrios Cardíacos/cirugía , Atrios Cardíacos/patología , Pronóstico , Fibrosarcoma/diagnóstico , Fibrosarcoma/cirugía , Neoplasias del Mediastino/patología
11.
J Cardiothorac Surg ; 19(1): 197, 2024 Apr 10.
Artículo en Inglés | MEDLINE | ID: mdl-38600499

RESUMEN

BACKGROUND: Left atrial myxoma during pregnancy is rare. We present three cases in order to aid in the management. CASE PRESENTATION: Three cases of left atrial myxoma during pregnancy were presented in this article. Three patients all received multidisciplinary team work and acquired good outcomes. The case 1 had no symptoms and delivered before traditional cardiac surgery. The case 2 and case 3 undergone totally endoscopic minimally invasive cardiac surgery during pregnancy. The case 3 maintained pregnancy to term and gave birth to a healthy baby via vaginal delivery. No relapse of the tumor was observed. CONCLUSIONS: The management of left atrial myxoma during pregnancy ought to be individualized and combined with the gestational age. If the diagnosis was made in the first two trimesters of pregnancy, totally endoscopic minimally invasive cardiac surgery during pregnancy would be an optimal choice. The patients can benefit from the multidisciplinary team work.


Asunto(s)
Neoplasias Cardíacas , Mixoma , Humanos , Embarazo , Femenino , Mujeres Embarazadas , Atrios Cardíacos/cirugía , Recurrencia Local de Neoplasia , Neoplasias Cardíacas/diagnóstico , Neoplasias Cardíacas/cirugía , Mixoma/diagnóstico , Mixoma/cirugía
12.
J Cardiothorac Surg ; 19(1): 243, 2024 Apr 18.
Artículo en Inglés | MEDLINE | ID: mdl-38632629

RESUMEN

Synovial sarcoma of the heart is a rare tumor. Herein we would like to report a case of giant intrapericardial cardiac synovial sarcoma that originated from the right ventricle and grew outward near the diaphragm. After making adequate preoperative preparation, we performed the surgery as quickly as possible and resected the tumor completely. Based on the identification of the translocation on chromosome 18 rearrangement, the tumor can be diagnosed as a primary cardiac synovial sarcoma. Through this study, we aim to afford more information about cardiac synovial sarcomas as well as a reference for similar cases.


Asunto(s)
Neoplasias Cardíacas , Neoplasias del Mediastino , Sarcoma Sinovial , Neoplasias del Timo , Humanos , Sarcoma Sinovial/diagnóstico , Neoplasias Cardíacas/cirugía , Ventrículos Cardíacos/patología
13.
Praxis (Bern 1994) ; 113(3): 78-82, 2024 Mar.
Artículo en Alemán | MEDLINE | ID: mdl-38655734

RESUMEN

INTRODUCTION: Ischaemic stroke results from a sudden interruption of cerebral blood flow, often caused by thrombosis, embolism or hypoperfusion. Rarely, cardiac tumours are the cause of an embolic event. We report on an 80-year-old woman with a cerebral stroke. Successful thrombectomy revealed a histopathologically confirmed papillary fibroelastoma as the embolic source. Although it was not detectable on initial echocardiography, the tumour was discovered by transesophageal echocardiography. This case emphasises the importance of considering primary cardiac tumours as potential sources of emboli in ischaemic stroke. Keywords.


Asunto(s)
Ecocardiografía Transesofágica , Neoplasias Cardíacas , Humanos , Femenino , Anciano de 80 o más Años , Neoplasias Cardíacas/diagnóstico por imagen , Neoplasias Cardíacas/cirugía , Neoplasias Cardíacas/patología , Diagnóstico Diferencial , Trombectomía , Accidente Cerebrovascular Isquémico/diagnóstico por imagen , Accidente Cerebrovascular Isquémico/etiología , Accidente Cerebrovascular Isquémico/cirugía , Fibroma/cirugía , Fibroma/diagnóstico por imagen , Fibroma/patología
14.
Am J Case Rep ; 25: e942864, 2024 Apr 23.
Artículo en Inglés | MEDLINE | ID: mdl-38650318

RESUMEN

BACKGROUND We present a case of metachronous cardiac and intramuscular metastases in a patient with a known history of radical nephroureterectomy for upper-tract urothelial carcinoma (UTUC). CASE REPORT A 58-year-old man had a history of metachronous renal pelvis urothelial carcinoma with prior left radical nephroureterectomy. He was also diagnosed with malignancy-associated deep vein thrombosis (DVT) and was on rivaroxaban. He presented at an oncology follow-up consult with shortness of breath and right scapular lump. CT scan revealed a soft-tissue mass at the surgical bed suspicious for local recurrence, as well as intracardiac hypodensities and intramuscular nodules in the right latissimus dorsi and right adductor muscles. The intracardiac hypodensities were located in the left atrial appendage and inter-atrial septum. Given that the patient had a history of DVT and in a pro-thrombotic state, differentials for the intracardiac densities included intracardiac thrombi or metastases. The intramuscular hypodensities were rim-enhancing. Given that the patient was on rivaroxaban, differentials included hematomas or metastases. As there was no overlying bruising and the lesions remained unchanged in size clinically, they were treated as metastases. The patient was treated with clexane but re-presented with worsening of shortness of breath and palpitations. CT scan showed increased size of intracardiac lesions, suggesting no response to anticoagulation, and therefore were likely metastatic in nature. He completed a 2-year course of IV pembrolizumab and was in complete remission. CONCLUSIONS Our case highlights the importance of this clinically challenging scenario when patients with known malignancy and on anticoagulation present with cardiac or musculoskeletal symptoms. Though these patients are at risk of thrombus and haematoma, cardiac and intramuscular metastasis should be considered, as the prognosis is guarded.


Asunto(s)
Carcinoma de Células Transicionales , Neoplasias Cardíacas , Neoplasias Renales , Neoplasias de los Músculos , Nefroureterectomía , Humanos , Masculino , Persona de Mediana Edad , Neoplasias Cardíacas/secundario , Neoplasias Cardíacas/cirugía , Neoplasias de los Músculos/secundario , Carcinoma de Células Transicionales/secundario , Carcinoma de Células Transicionales/cirugía , Neoplasias Renales/patología , Neoplasias Primarias Secundarias , Trombosis de la Vena/etiología , Tomografía Computarizada por Rayos X
16.
Kyobu Geka ; 77(3): 222-225, 2024 Mar.
Artículo en Japonés | MEDLINE | ID: mdl-38465496

RESUMEN

We experienced a case of papillary fibroelastoma of aortic valve, which had been located for preoperative 12 years without any symptom. We could assess tumor growth rate of 0.17 mm/year. Because of recent reports of recurrence, close follow-up should be continued.


Asunto(s)
Fibroelastoma Papilar Cardíaco , Fibroma , Neoplasias Cardíacas , Humanos , Válvula Aórtica/diagnóstico por imagen , Válvula Aórtica/cirugía , Fibroelastoma Papilar Cardíaco/patología , Neoplasias Cardíacas/diagnóstico por imagen , Neoplasias Cardíacas/cirugía , Fibroma/diagnóstico por imagen , Fibroma/cirugía
18.
J Med Case Rep ; 18(1): 151, 2024 Mar 11.
Artículo en Inglés | MEDLINE | ID: mdl-38462621

RESUMEN

BACKGROUND: Myxomas are the most common primary cardiac tumor and typically originate in the left atrium. Atrial myxomas may present following complications of obstruction and emboli. If an atrial myxoma goes untreated, complications such as congestive heart failure, embolic stroke, and sudden death can occur. CASE PRESENTATION: A 58-year-old Caucasian male presented following a cardiac arrest. He was taken emergently to the cardiac catheterization lab and received two drug eluting stents. Following the procedure, he was found to have a left atrial mass that was intermittently obstructing the mitral valve on echocardiography. After leaving the cardiac catheterization lab, he was hypotensive and placed on multiple intravenous medications for hemodynamic support as well as an Impella device. Following medical optimization, he underwent one vessel coronary artery bypass graft as well as surgical excision of the left atrial mass, which pathology had shown to be an atrial myxoma. CONCLUSION: This patient's case of cardiogenic shock following revascularization was complicated by the identification of an atrial myxoma, which, when large enough, can obstruct blood flow through the mitral valve leading to acute mitral dynamic stenosis. This condition results in circulatory collapse due to obstruction of the left ventricle in diastole as the myxoma occludes the mitral valve.


Asunto(s)
Neoplasias Cardíacas , Infarto del Miocardio , Mixoma , Humanos , Masculino , Persona de Mediana Edad , Choque Cardiogénico/etiología , Infarto del Miocardio/complicaciones , Ecocardiografía , Atrios Cardíacos/diagnóstico por imagen , Neoplasias Cardíacas/complicaciones , Neoplasias Cardíacas/diagnóstico por imagen , Neoplasias Cardíacas/cirugía , Mixoma/complicaciones , Mixoma/diagnóstico por imagen , Mixoma/cirugía
19.
J Cardiothorac Surg ; 19(1): 161, 2024 Mar 28.
Artículo en Inglés | MEDLINE | ID: mdl-38549142

RESUMEN

BACKGROUND: Primary cardiac angiosarcomas are very rare and present aggressively with high rates of metastasis. Given the poor prognosis, particularly once disease has spread, early diagnosis and multidisciplinary treatment is essential. CASE PRESENTATION: We present the case of a 46-year-old male who presented with chest pain, intermittent fevers, and dyspnea. Workup with computed tomography scan and transesophageal echocardiography demonstrated a right atrial pseudoaneurysm. Given the concern for rupture, the patient was taken to the operating room, where resection of the pseudoaneurysm and repair using a bovine pericardial patch was performed. Histopathology report initially demonstrated perivascular lymphocyte infiltrate. Six weeks later, the patient represented with chest pain and new word finding difficulty. Workup revealed multiple solid lung, pericardial, brain, and bone nodules. Eventual biopsy of a cardiophrenic nodule demonstrated angiosarcoma, and rereview of the original pathology slides confirmed the diagnosis of primary cardiac angiosarcoma. CONCLUSIONS: Primary cardiac angiosarcomas are often misdiagnosed given the rarity of these tumors, but early diagnosis and initiation of treatment is essential. The unique presentation of our case demonstrates that clinical suspicion for cardiac angiosarcoma should be maintained for spontaneous pseudoaneurysm originating from the right atrium.


Asunto(s)
Aneurisma Falso , Neoplasias Cardíacas , Hemangiosarcoma , Neoplasias del Mediastino , Neoplasias del Timo , Masculino , Humanos , Animales , Bovinos , Persona de Mediana Edad , Hemangiosarcoma/diagnóstico , Hemangiosarcoma/cirugía , Diagnóstico Tardío , Atrios Cardíacos/cirugía , Atrios Cardíacos/patología , Neoplasias Cardíacas/diagnóstico , Neoplasias Cardíacas/cirugía , Neoplasias Cardíacas/patología , Neoplasias del Mediastino/patología , Neoplasias del Timo/patología , Dolor en el Pecho
20.
Pediatr Cardiol ; 45(5): 1132-1141, 2024 Jun.
Artículo en Inglés | MEDLINE | ID: mdl-38480570

RESUMEN

Cardiac rhabdomyomas are the most common benign pediatric heart tumor in infancy, which are commonly associated with tuberous sclerosis complex (TSC). Most rhabdomyomas are asymptomatic and spontaneously regress over time. However, some cases especially in neonates or small infants can present with hemodynamic instability. Surgical resection of the tumor, which has been the gold standard in alleviating obstruction, is not always possible and may be associated with significant morbidity and mortality. Recently, mammalian target of rapamycin inhibitors (mTORi) have been shown to be safe and effective in the treatment of TSC. We present the outcomes of neonates and an infant who received treatment for symptomatic rhabdomyomas at a tertiary cardiology center. Medical records were reviewed to obtain clinical, demographic, and outcome data. Six patients received interventions for symptomatic rhabdomyomas, median age at presentation was 1 day old (range from 1 to 121 days old), and 67% of the patients had a pathogenic mutation in TSC gene. One patient underwent surgical resection of solitary tumor at right ventricular outflow tract (RVOT) successfully. In the four patients with left ventricular outflow tract (LVOT) obstruction, two patients received combined therapy of surgical debulking of LVOT tumor, Stage I palliation procedure, and mTORi and two patients received mTORi therapy. One patient with RVOT obstruction underwent ductal stenting and received synergistic mTORi. Four of the five patients had good response to mTORi demonstrated by the rapid regression of rhabdomyoma size. 83% of patients are still alive at their latest follow-up, at two to eight years of age. One patient died on day 17 post-LVOT tumor resection and Hybrid stage one due to failure of hemostasis, in the background of familial factor VII deficiency. Treatment of symptomatic rhabdomyoma requires individualized treatment strategy based on the underlying pathophysiology, with involvement of multidisciplinary teams. mTORi is effective and safe in inducing rapid regression of rhabdomyomas. A standardized mTORi prescription and monitoring guide will ensure medication safety in neonates and infants with symptomatic cardiac rhabdomyoma. Although the majority of tumors responded to mTORi, some prove to be resistant. Further studies are warranted, ideally involving multiple international centers with a larger number of patients.


Asunto(s)
Neoplasias Cardíacas , Rabdomioma , Obstrucción del Flujo Ventricular Externo , Humanos , Neoplasias Cardíacas/terapia , Neoplasias Cardíacas/cirugía , Neoplasias Cardíacas/complicaciones , Rabdomioma/complicaciones , Rabdomioma/cirugía , Rabdomioma/diagnóstico , Rabdomioma/terapia , Lactante , Recién Nacido , Masculino , Femenino , Obstrucción del Flujo Ventricular Externo/etiología , Obstrucción del Flujo Ventricular Externo/terapia , Obstrucción del Flujo Ventricular Externo/cirugía , Estudios Retrospectivos , Resultado del Tratamiento , Ecocardiografía , Esclerosis Tuberosa/complicaciones , Esclerosis Tuberosa/terapia , Esclerosis Tuberosa/diagnóstico , Procedimientos Quirúrgicos Cardíacos/métodos , Inhibidores mTOR/uso terapéutico
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