Periodontitis in marfan syndrome: a case report

A Síndrome de Marfan é uma doença autossômica dominante do tecido conjuntivo, caracterizada por alterações nos sistemas cardiovascular, esquelético e ocular, e que pode aumentar a suscetibilidade à doença periodontal. Esse relato de caso descreve dados periodontais clínicos, microbiológicos e imunológicos de um paciente de 28 anos, gênero masculino, com diagnóstico clínico de Síndrome de Marfan. Neste caso, as principais alterações estão nos sistemas esquelético e ocular. A principal alteração intraoral é a presença de palato profundo e prognatismo mandibular. No exame clínico periodontal, a média do nível clínico de inserção foi de 2,35 mm e índice de sangramento à sondagem de 30%. O tratamento periodontal foi executado em uma sessão de debridamento e orientação de higiene oral, sob antibioticoterapia profilática. Na reavaliação, o paciente apresentou melhora nos parâmetros clínicos periodontais. O relato de caso apresenta um paciente com alterações leves, que afetam a saúde bucal. Em casos de Síndrome de Marfan, a manutenção da saúde periodontal é essencial para um bom prognóstico da saúde bucal.(AU)

Marfan syndrome is an autossomal dominant disorder of connective tissue characterized by alteration in cardiovascular, skeletal and ocular system, and may increase the susceptibility of periodontal disease. This case report describes the clinical, microbiological and immunological periodontal findings in a 28 year old male patient with a clinical diagnosis of Marfan syndrome. The major alterations of the case were in ocular and skeletal system. The major oral alterations were the high arched and narrow palate, and mandibular prognathism. At periodontal examination, an average clinical attachment level loss of 2.35 mm and 30% of bleeding on probing were found. The periodontal treatment was performed, in one session of periodontal debridement with prophylactic antibiotic premedication and oral hygiene instructions. At the revaluation, the patient showed improved clinical parameters. This case report presented a patient with mild features of a genetic disorder which affects oral health. The maintenance of periodontal health in Marfan syndrome cases is essential for a favorable prognosis of oral health.(AU)

Anestesia para gestante cardiopata / Anaesthesia for pregnant cardiac disease

Gestantes com doença cardíaca habitualmente possuem prognóstico favorável tanto materno quanto fetal. Com exceção das pacientes com a síndrome de Eisenmenger, hipertensão pulmonar primária e síndrome de Marfan com aortopatia, morte materna durante a gravidez em pacientes cardiopatas é rara. A gravidez por si só impõe modificações hemodinâmicas significativas, colocando à prova o sistema cardiovascular. Doença cardíaca reumática é a mais frequente nas gestantes, e o edema agudo pulmonar, a complicação mais comum. Defeito do septo atrial é a cardiopatia congênita acianótica mais prevalente na população adulta, enquanto que a Tetralogia de Fallot é a mais frequente das cardiopatias congênitas cianóticas. Gravidez e cardiopatia são uma associação de grandes desafios para o anestesiologista. Para evitar complicações decorrentes da morbidade ou mortalidade materno-fetal, o anestesiologista deve conhecer a evolução da doença durante a gravidez. Aqui são discutidas a fisiopatologia, apresentação clínica e a condução anestésica das doenças cardíacas valvulares adquiridas, das doenças cardíacas congênitas, da doença isquêmica do miocárdio e das miocardiopatias na gravidez.

Pregnancy in most women with heart disease has a favorable maternal and fetal outcome. With the exception of patients with Eisenmenger syndrome, pulmonary hypertension primary, and Marfan syndrome with aortopathy, maternal death during pregnancy in women with heart disease is rare. Pregnancy per se imposes significant hemodynamic changes placing a major burden on the cardiovascular system. Rheumatic heart disease remains the most frequent heart disease in the pregnant population and the pulmonary edema is the most frequent complication. Atrial septal defect is the most frequent congenital acianotic heart disease in the adult population, whereas tetralogy of Fallot is the most common cyanotic congenital heart disease. Pregnancy and heart disease present a unique challenge to the anesthesiologist. To avoid untoward complications resulting in significant maternal and/or fetal morbidity or mortality, the anesthesiologist must be familiar about the progression of heart disease during pregnancy. In this article, we review the pathophysiology, clinical presentation, and anesthetic management of valvular, congenital, vascular and ischemic heart disease, and cardiomyopathy in pregnancy.

Beta-blocker therapy does not alter the rate of aortic root dilation in pediatric patients with Marfan syndrome.

OBJECTIVE: To test the hypothesis that chronic beta-blocker therapy in pediatric patients with Marfan syndrome alters the rate of aortic root dilation. Beta-blockade has been advocated as preventive therapy for Marfan syndrome based on reports indicating a decreased rate of aortic root dilation in treated patients. STUDY DESIGN: Patients with Marfan syndrome (n = 63) followed at Children's Hospital of Pittsburgh or Children's Hospital of New York-Presbyterian who had > or =18 months of echocardiographic follow-up were studied. All clinical data and 213 serial echocardiograms were reviewed, and aortic root dimensions were measured. Patients were divided into 2 groups for comparison: untreated (n = 34) and treated (n = 29). RESULTS: At study entry, the 2 study groups were comparable in terms of age, sex, body surface area (BSA), aortic root measurements, heart rate, and corresponding z scores. Follow-up duration in each group was similar. At last follow-up, heart rates and heart rate z scores were lower in the treated group. Rates of change of aortic root measurements (P = .52) and the corresponding z scores were not statistically different between the 2 group at the study's end. CONCLUSIONS: This study suggests that that beta-blocker therapy does not significantly alter the rate of aortic root dilation in children with Marfan syndrome. Based on these data, the recommendation of lifetime beta-blocker therapy instituted during childhood should be reassessed.