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Twenty Years of Clinical Experience with Cardiac Myxomas: Diagnosis, Treatment, and Follow Up.

ABSTRACT: BACKGROUND:Cardiac myxomas are the most frequent cardiac tumors. Although histologically benign, in some cases myxomas may be lethal, due to impairment of cardiac dynamics and their thromboembolic potential. The study aimed to assess the clinical presentation of cardiac myxomas and their correlation with echocardiographic features and to describe the perioperative results and long-term outcome of surgically treated patients. METHODS:A prospective study of 53 patients with cardiac myxomas who were operated the Hospital Argerich, followed clinically and with echocardiography from 1993 until 2013. All patients underwent echocardiographic studies. RESULTS:The patient's mean age was 53 ± 16 years (62.3% were women). The most common findings were dyspnea followed by embolic events. Most tumors were localized in the left atrium (77.4%), mainly in the fossa ovalis (63%). Mean size of the tumors was 4.76 x 3.50 cm. Tumors were generally mobile (88%) and went beyond the valve plane, causing mild mitral or tricuspid valve obstruction (58%) and dilation of the respective atrial chamber. Patients whose tumors were obstructive had higher pulmonary artery systolic pressures (50 vs 33 mmHg p < 0.01). According to the echocardiographic appearance 67% of tumors had a smooth surface and the remaining 32% had a villous surface. All patients with embolic manifestations had tumors with a villous surface. CONCLUSIONS:Clinical presentation relates to the ultrasound characteristics of myxomas. Smooth tumors are larger, occur with obstructive symptoms, and benefit from an elective surgery, whereas villous myxomas entailed a high embolic risk and require prompt surgical treatment.

SUBMITTER: Cianciulli TF

PROVIDER: S-EPMC6358422 | biostudies-literature | 2019 Jan

REPOSITORIES: biostudies-literature

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Twenty Years of Clinical Experience with Cardiac Myxomas: Diagnosis, Treatment, and Follow Up.

Cianciulli Tomás Francisco TF Cozzarin Alberto A Soumoulou Juan Bautista JB Saccheri María Cristina MC Méndez Ricardo José RJ Beck Martín Alejandro MA Gagliardi Juan Alberto JA Lax Jorge Alberto JA

Journal of cardiovascular imaging 20190101 1

<h4>Background</h4>Cardiac myxomas are the most frequent cardiac tumors. Although histologically benign, in some cases myxomas may be lethal, due to impairment of cardiac dynamics and their thromboembolic potential. The study aimed to assess the clinical presentation of cardiac myxomas and their correlation with echocardiographic features and to describe the perioperative results and long-term outcome of surgically treated patients.<h4>Methods</h4>A prospective study of 53 patients with cardiac ...[more]

PMID: 30701715

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Project description:IntroductionBiotinidase deficiency (BTD) is an inborn error of biotin metabolism inherited as an autosomal recessive trait. Due to the, biotinidase deficiency, biotin is not recycled. Individuals with BTD usually exhibit neurological and cutaneous abnormalities unless treated with biotin. Supplementation with biotin may either ameliorate or if early introduced even prevent symptoms when introduced presymptomatically.Patients and methodsSince 1991, 22 Polish patients from 19 families have been diagnosed with BTD. In 16 children the diagnosis had been suspected on the basis of clinical signs: skin lesions, hyperventilation, seizures, spasticity, and laboratory investigation (elevated lactate and metabolites on urine organic acids profile). The defect was enzymatically (serum biotinidase activity measurement) and genetically (tested for mutations in the BTD gene) confirmed afterwards. All patients were treated with biotin. Urine organic acids analysis (GC/MS) for 3-hydroxizovaleric acid was used for patients' monitoring. Neurological, audiological and ophthalmological evaluation has been conducted once a year.ResultsIn 5 symptomatic patients a progressive optic nerve atrophy had already been noted at the time of treatment initiation. In these patients sensorineural hearing loss has also been diagnosed despite biotin supplementation. Asymptomatic patients treated with biotin supplementation presented no signs or symptoms of BTD. Supplementation with biotin slows the progression of BTD in symptomatic patients, but does not reverse nerve atrophy. Nonetheless, introduction of the treatment with biotin during presymptomatic stage of the disease prevents the onset of symptoms including optic atrophy and hearing loss. Homozygosity for the p.Leu215Phe mutation in BTD gene seems to be frequent in patients from the North-Eastern region of Poland and is connected with the hearing loss.ConclusionSince the prognosis for individuals diagnosed with BTD is good, provided they are treated before symptoms occur, it is justified to add this metabolic disorder to the panel of conditions screened under the national newborn screening programme in Poland.

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Twenty Years of Clinical Experience with Cardiac Myxomas: Diagnosis, Treatment, and Follow Up.

Publications

Twenty Years of Clinical Experience with Cardiac Myxomas: Diagnosis, Treatment, and Follow Up.

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