Congenital Atrial Haemangioma

Introduction Primary cardiac tumours are rare. We report the first known case of congenital cardiac haemangioma in Ireland. Case A neonate presented with symptoms arising from a congenital atrial haemangioma on day three of life. The mass was successfully excised via median sternotomy and bicaval cannulation for cardiopulmonary bypass. The patient was discharged day ten postoperatively and remained well at one year follow-up. Discussion The degree of debulking surgery required varies depending on tumour type. This report serves to aid clinicians in accurately suspecting, investigating and diagnosing patients with cardiac tumours.

Intra-cardiac Tumor in Newborn: An Echo Presentation.

Cardiac tumors represent a relatively rare, yet challenging diagnosis. Echocardiography is the main diagnostic tool for the detection of a cardiac tumor. We report a case of congenital giant intra-cardiac tumor, in which echocardiography revealed a large well-defined hyperechoic homogenous intracardiac mass involving interventricular septum which mimicked like asymmetrical septal hypertrophic pattern and another two small masses attached to right ventricular part of interventricular septum.

Successful resection of a primary cardiac fibroma in a neonate: report of a case.

During the fetal-neonatal period, a primary cardiac tumor may be completely asymptomatic and such tumors may be incidentally discovered by echocardiography. A four-hour-old male was diagnosed to have a cardiac tumor by post-natal echocardiography and was observed closely. Surgery was indicated immediately at the 3 week follow-up examination when the tumor was found to have obstructed the right ventricle outflow. The tumor was resected successfully and its histopathology indicated that it was a fibroma. Follow-up echocardiograms and magnetic resonance imaging 5 months postoperatively demonstrated no evidence of any remaining tumor and his RV function was good.

[Cardiac papillary fibroelastoma on the Chiari's network of a 3-month-old baby; report of a case].

A 3-month-old boy was transferred to our hospital because of a cardiac tumor in the right heart, which had been prenatally diagnosed by fetal echocardiography. During the operation, the tumor was found on the Chiari's network in the right atrium. The tumor was excised and the ventricular septal defect was repaired. Postoperative histological examination revealed that the tumor was a papillary fibroelastoma. He was transferred to a pediatric unit for postoperative care.

A case of neonatal intrapericardial teratoma. Clinical and pathological findings.

AIM: It is of general agreement that complete surgical removal after birth of intrapericardial fetal teratomas is needed, because of the risk of severe cardiovascular and respiratory distress, related to the mass size, location and secondary pericardial effusion. Histological examination generally shows mature aspect of cells and tissues. METHODS: We present a case of grade II immature pericardial teratoma, diagnosed in utero and completely removed after birth. RESULTS: Even surgical removal was complete, histological aspects raised the need of long follow-up with serial alpha-fetoprotein determinations. CONCLUSION: A neonatal grade II immature pericardial teratoma was completely removed after birth. The follow-up of the patient, until 10 months of life, was good with no recurrence of the disease.

Large multifocal cardiac myxoma causing the sudden unexpected death of a 2-month-old infant--a rapidly growing, acquired lesion versus a congenital process?: a case report.

We report the occurrence of a clinically undiagnosed biatrial myxoma with left ventricular involvement in a 2-month-old male infant, resulting in sudden death. During a routine well-baby examination, a grade (34) holosystolic murmur was detected at the left sternal border with radiation to the axilla and back. On the following day, the patient collapsed and died suddenly. An autopsy revealed a large multifocal neoplasm diffusely involving the aortic valve while displaying mitral, tricuspid, and left ventricular extensions. The ensuing histopathologic and immunohistochemical studies were diagnostic for myxoma. We discuss the occurrence of cardiac myxoma within the pediatric population and review the literature as to theorize whether this lesion was a congenital process versus a rapidly growing tumor that developed after the child was born. Lastly, we address the potential for sudden death in patients with such tumors.

Intrauterine fetal death due to cardiac rhabdomyomas.

A 21-year-old patient, in her first and regularly controlled uneventful pregnancy, was admitted to hospital due to lower leg edema, hypertension, proteinuria, and weight gain. Fetal death occurred the next day and a female nonhydropic fetus, 40 cm CH, 1460 grams, at 29-week gestation was delivered. An autopsy showed no visible gross abnormalities except in the heart. The heart was enlarged, with five intramural and subendocardial nodules, 0.3 to 1 cm in size, three in the left ventricular free wall, and one in the right ventricle and right atrium, sharply demarcated, reddish-gray, moderately firm, with the typical appearance of rhabdomyoma.

Intrapericardial teratoma as a cause of fetal death--a case report.

Intrapericardial teratoma is a rare congenital tumor that without treatment leads to cardiac failure in either the prenatal or postnatal period. Early diagnosis and recent surgical advances can, in some cases, delay development of intrauterine symptoms and allow final treatment through a tumor resection. However, a large number of intrapericardial tumors go undetected during prenatal diagnostics, until they are found as a cause of intrauterine death or postnatal cardiorespiratory insufficiency, as in our case report. An abortion was induced in the 23rd gestational week because there was no cardiac activity detected during a routine ultrasound scan in a 35-year old woman. The tumor was found during the postmortem of the fetus.

A curious isolated cystic lesion of the membranous atrioventricular septum.

We present the case of an isolated cystic lesion of the atrioventricular component of the membranous septum of unclear aetiology, but responsible for cardiomegaly and benign disturbances of cardiac rhythm. As far as we are aware, this type of lesion has not previously been documented.

The Use of Video-Assisted Cardioscopy for Neonatal Left Ventricular Tumor Resection.

We report the use of video-assisted cardioscopy (VAC) to assess the complete resection of a giant sessile rhabdomyoma of the left ventricle (LV) in a case of suspected neonatal tuberous sclerosis. A fetal echocardiogram performed at 20 weeks of gestation identified the mass at the apex of the LV and attached to the interventricular septum (IVS). Further echocardiography during the pregnancy demonstrated moderate growth of the LV mass. This was occupying more than two-thirds of the LV cavity after the delivery, and there were concerns of LV outflow tract obstruction. The 4-kg newborn underwent LV tumor excision via a left atrial approach at the age of 12 days. The mitral valve (MV) apparatus and the IVS had strong attachments to the mass. The tumor was successfully resected. In view of the invasiveness of the tumor, a decision was taken to perform an intracardiac assessment using a VAC to detect any iatrogenic complication. A Karl Storz Neuro Endoscope probe 4 mm in diameter and 14 mm in length with an angulation of 0° was passed into the left ventricular cavity through the MV with the aim to detect residual tumor and to identify any iatrogenic complications due to the extensive resection. Video-assisted cardioscopy allows visualization of inaccessible intraventricular structures avoiding ventriculotomy, which can cause ventricular dysfunction, arrhythmias, and aneurysm formation. The VAC was shown to be very useful in this situation, and the process can easily be repeated as required.

Management of Complications Caused by a Massive Left Ventricle Tumor in a Neonate.

We report the case of a neonate born with a giant fibroma occupying the entirety of her left ventricle. Owing to the extensive resection, her postoperative course was complicated by severely diminished left ventricular function and complete heart block necessitating extracorporeal support. Ultimately, cardiac resynchronization therapy was used, after which the infant's ventricular function gradually improved and she was successfully discharged to home.

Heart tumors in children and adults: clinicopathological study of 59 patients from a surgical center.

BACKGROUND: Heart tumors are rare lesions with variegated histological types. Their clinicopathological features could be more comprehensively categorized. METHODS: This is a 19-year retrospective study of 17 infants/toddlers (<2 years of age) and 42 patients aged between 14 and 79 years (mean = 51.5) in a surgical center. RESULTS: Congenital tumors (n = 17; 29%), including rhabdomyomas (n = 9), ventricular fibromas (n = 6), and hemangiomas (n = 1), required surgery mainly because of mass effect. Familial myofibromatosis was the only embolic congenital lesion. Acquired benign tumors (n = 28; 47%) included myxomas (n = 21), fibroelastomas (n = 3), myofibroblastic inflammatory tumors (n = 2), and lipomas (n = 2). Eight (29%) were revealed by systemic embolization. These benign noncongenital tumors were all treated by complete resection, except for an incompletely resected lipoma of the mitral valve. Postoperative arrhythmia (n = 1) and pericardial effusion (n = 3) were the only complications. Primary sarcomas (n = 8; 14%) were mostly vascular tumors (five of eight), and patients with high-grade tumors had a mean survival of 15 months (n = 5). Cardiac metastases (n = 6; 10%) were from carcinomas (n = 3) or sarcomas (n = 3); apart from a necrotic metastasis, all patients died (mean survival of 6 months). CONCLUSIONS: This study shows that, regardless of patients' age, heart tumors can be classified as: (a) congenital lesions, which are spontaneously nonprogressive or regressive lesions possibly requiring surgery mainly because of mass effect; (b) acquired benign tumors, which are lesions requiring surgery often because of embolization risk; and (c) primary and secondary malignant tumors, which are lesions with globally poor prognosis but with some indications for resection.

Benign congenital intracardiac thyroid and polycystic tumor causing right ventricular outflow tract obstruction and conduction disturbance.

A patient with a rare intracardiac tumor arising from the right side of the interventricular septum who developed conduction disturbances and symptoms of right ventricular outflow tract obstruction is reported. The patient was successfully treated with insertion of a permanent pacemaker and surgical removal of the tumor after two-dimensional echocardiography diagnosed the intracardiac mass. Pathologic examination confirmed the benign nature of the tumor and showed the unusual features of both ectopic thyroid and benign congenital polycystic tumor.

Primary right ventricular tumor (fibroma) simulating cyanotic heart disease in a newborn.

The clinical, echocardiographic, cineangiocardiographic and autopsy findings in a newborn infant with right ventricular fibroma are reported. The tumor caused severe right ventricular outflow obstruction simulating cyanotic heart disease. Echocardiography allowed a definitive diagnosis of the tumor mass at 10 hours of age. The clinical implications of the case are discussed.

Investigation and management of primary cardiac tumors in infants and children.

Seven children with primary cardiac tumors are discussed. The diagnosis was achieved by cardiac catheterization in all seven patients. In four patients, surgical excision was undertaken with one long-term good result. One of the other three surgical patients died of acute heart failure at the discontinuation of cardiopulmonary bypass. One of the other two surgical patients died suddenly late postoperatively, presumably from arrhythmia. The other, affected by fibrosarcoma, also died suddenly while undergoing chemotherapy. The histologic findings on the tumor masses were available in six patients. There were three rhabdomyomas, one fibrosarcoma, one fibroma and one hamartomatous mass. In one patient with tuberous sclerosis, a presumptive diagnosis of rhabdomyoma was made clinically on the basis of multiple masses infiltrating the left ventricle. Two-dimensional echocardiography is the best noninvasive tool to determine the extent and location of the tumor and suitability for surgery. When surgery is indicated, cardiac catheterization for pressure data is also required. Surgery is recommended in symptomatic solitary lesions that most likely are fibromas and have an overall good surgical outcome.

Intraoperative cardiac mapping in the treatment of an infant congenital fibroma.

Surgical treatment for ventricular tachycardia associated with congenital cardiac tumors is rare. Intraoperative electroanatomic mapping was performed in a 23-month-old female infant to identify the arrhythmogenic substrate of the epicardium before tumor resection. Verification of the localized abnormal electrocardiogram on the tumor in the treatment of ventricular tachycardia was useful for successful partial resection and cryoablation of the giant fibroma.

Association between cardiac tumors and tuberous sclerosis in the fetus and neonate.

A retrospective review was performed in 94 patients with > or =1 cardiac tumors seen on prenatal or neonatal echocardiography at 5 major referral centers. Tuberous sclerosis was present in 68 patients diagnosed with a cardiac tumor in utero or during the neonatal period, including 61 of 64 with multiple tumors.

Congenital papillary tumor of the tricuspid valve. An unusual cause of right ventricular outflow obstruction in a neonate with trisomy E.

A trisomy E neonate had congenital cardiac anomalies that included a ventricular septal defect, a bicuspid aortic valve, and a congenital papillary tumor of the tricuspid valve. The large papillary tumor was responsible for severe intermittent pulmonary outflow obstruction. The cause and pathogenesis of these rare papillary tumors are unknown, but they are probably related to the more sessile varieties of congenital valvular dysplasia. The life-threatening obstruction to blood flow caused by the congenital valvular tumor probably can be alleviated by simple surgical resection. This type of tumor should be considered in the differential diagnosis of atypical forms of outflow tract obstruction.