Avaliacão clínico-cardiológica e ecocardiográfica, seqüencial, em criancas portadoras da síndrome de Marfan / Serial clinical and echocardiographic evaluation in children with Marfan syndrome

OBJETIVO: Descrever a apresentacão clínica cardiológica e a evolucão temporal, estimar a incidência de ectasia ânulo-aórtica e de prolapso da valva mitral, e avaliar a tolerância e a efetividade dos betabloqueadores em criancas com síndrome de Marfan. MÉTODOS: Foram submetidas a exame clínico e ecocardiográfico seriado, durante um ano, 21 criancas com síndrome de Marfan. No ecocardiograma foram analisados: presenca de prolapso mitral, diâmetro da raiz aórtica, refluxos das valvas mitral e aórtica, e o crescimento dos diâmetros aórticos na vigência de betabloqueadores. Em 11 pacientes foi possível obter duas medidas da raiz aórtica no intervalo de um ano. RESULTADOS: Durante o estudo as criancas não apresentaram sintomas. Prolapso mitral foi encontrado em 11 (52 por cento) criancas. Ectasia ânulo-aórtica ocorreu em 16 (76 por cento) pacientes, sendo de grau discreto em 42,8 por cento, moderado em 9,5 por cento, e importante em 23,8 por cento. Um desses pacientes foi submetido com sucesso à cirurgia de Bentall DeBono. Com o uso de betabloqueador a freqüência cardíaca diminuiu 13,6 por cento (de 85 para 73 bpm; p < 0,009), mas houve um crescimento da raiz aórtica de 1,4 mm/ano (p < 0,02). Uma crianca não pôde receber betabloqueador em razão de asma brônquica, e não foram observados efeitos colaterais significativos nas outras criancas, incluindo uma com asma brônquica. CONCLUSAO: Os resultados obtidos sugerem que, no período observado, as criancas permaneceram assintomáticas, o uso de betabloqueadores diminuiu significativamente a freqüência cardíaca e não se acompanhou de efeitos adversos significativos. Ao contrário da literatura, a incidência de ectasia ânulo-aórtica foi elevada e maior do que a de prolapso valvar mitral, tendo crescimento mesmo na vigência de uso eficaz de betabloqueador.

Manejo de la insuficiencia aórtica

La insuficiencia aórtica es una valvulopatía producida por múltiples entidades que van a desencadenar un proceso fisiopatológico común, el cual finalmente producirá daño miocárdico, aparición de síntomas y desarrollo de mortalidad. El manejo de esta patología va dirigido inicialmente a retardar el desarrollo de daño miocárdico con el uso de vasodilatadores, pero una vez este se ha desarrollado o, el paciente presenta síntomas, se debe realizar el tratamiento definitivo que corresponde al cambio valvular aórtico. El médico debe estar en capacidad de realizar un adecudo seguimiento para detectar precozmente los indicios del desarrollo de disfunción ventricular izquierda con el fin de indicar el reemplazo valvular pero, si dicha disfunción ya se ha desarrollado no se debe contraindicar por este solo motivo el tratamiento quirúrgico

Acute haemodynamic effects of nicorandil in patients with chronic severe regurgitant valvular lesions.

The haemodynamic effects of nicorandil, a new balanced vasodilator exhibiting nitrate-like as well as potassium-channel opening activity in patients with chronic severe valvular lesions have not been reported. We studied the acute effect of nicorandil on haemodynamics in 12 stable patients (6 males, 6 females; mean age 23.5 +/- 4.6 years) with chronic severe valvular regurgitation (8 mitral, 4 aortic). All patients were studied in resting, supine and fasting states. All cardioactive drugs were withdrawn five days prior to the study. Intra-arterial line was placed and thermodilution catheter was positioned in the pulmonary artery. Haemodynamic parameters recorded at baseline and at 30, 60, 90 and 120 minutes following a single oral dose of 20 mg nicorandil revealed no significant change in the heart rate while systemic pressures showed a small decline (p < 0.05). There was significant reduction in systolic, diastolic and mean pulmonary artery pressures (p < 0.001). The mean cardiac index increased from 3.16 L/min/m2 at baseline to 3.77 L/min/m2 at 60 minutes. Both the pulmonary and systemic vascular resistance indices reduced significantly, the peak fall being 18 percent and 29 percent, respectively. Maximal changes were observed at 60 to 90 minutes following administration of nicorandil. No adverse effect of nicorandil occurred during the study. We conclude that nicorandil has a favourable acute haemodynamic effect in patients with chronic severe valve regurgitation. Its long-term use in valvular lesions should be explored further.

Short-term evaluation of captopril in patients with chronic left sided valvular regurgitations.

OBJECTIVE: To evaluate the clinical effects and the changes in cardiac performance of high- and low-dose captopril compared to placebo in patients with chronic symptomatic aortic regurgitation (AR), and/or mitral regurgitation (MR). PATIENTS AND METHODS: We randomized patients into three groups, placebo (Group 1), incremental daily doses of 50 mg (Group 2), and 100 mg captopril (Group 3). We compared exercise capacity before and after four-week of treatment. RESULTS: Treatment was well tolerated with no serious side effects including blood chemistry. There were no significant effects of treatment on left ventricular dimensions nor calculated left ventricular ejection fraction (LVEF) between groups (LVEF change -0.6%, -2.6%, 2.4%, in group 1, 2 and 3 respectively; p > 0.05). No difference of exercise duration between treatment and placebo arms (change by 13%, 12.8%, 16.4%, respectively; p > 0.05). However, there were trends in the number of the patients who improved in left ventricular performance (absolute LVEF change > 5% unit = 15%, 16%, and 42% respectively; p > 0.05) and exercise performance (exercise time improvement > 75 sec = 50%, 47%, and 68% respectively; p > 0.05) in high dose captopril treatment group. CONCLUSION: There was no significant improvement of left ventricular performance and exercise capacity after four-weeks' treatment of low and high dose captopril. Further study with a larger sample size, and longer follow-up period may be required.

La digoxina es efectiva en el tratamiento de pacientes con insuficiencia cardíaca y ritmo sinusal / Digoxin is effectiveness in the treatment of cardiac failure and sinus rhythm patients

Due to differences in treatment effect in studies on the effectiveness of digoxin in patients with congestive heart failure in sinus rhythm, a cross-over placebo-controlled randomized double blind clinical trial was performed. Thirty one patients, without previous treatment with digoxin, in New York Heart Association (NYHA) functional class II to IV, with a dilated left ventricle and/or ventricular systolic dysfunction were included. Patients received digoxin, adjusted for blood levels, or placebo, during an 8 week period, prior to crossing over to the other treatment for another 8 weeks. The order of tretments was randomly allocated. Outcome measurement were performed at the end of each 8 week period. Digoxin, compared with placebo, improved NYHA class, 6,9 por ciento vs 41.4 por ciento (p=0.013) and increased the treadmill exercise time, 406 ñ 204 s vs 484 ñ 185 s (p=0.003). During the digoxin treatment the left ventricular and systolic diameter was reduced from 52.9 ñ 8.9 to 50.1 ñ 9.7 mm (p=0.009). No significant difference was observed in the left ventricular end diastolic diameter (LVED) of the left ventricle and in a estimation of quality of life. In conclusion, digoxin treatment produced a significant improvement in functional capacity, exercise time and left ventricular performance

Afterload reduction with captopril in chronic aortic regurgitation

The chronic hemodynamic effects of captopril were assessed in 22 asymptomatic patients with a moderate to severe isolated aortic regurgitation [AR] before and 3 months after administration of captopril [50-100 mg/day]. Supine heart rate decreased from 81.8 +/- 13.9 to 74.8 +/- 11.3 beats/min. [P <0.01], supine systolic blood pressure from 143.16 +/- 11.07 to 118.18 +/- 9.2 mmHg [P <0.001], mean wall stress from 796.77 +/- 162.78 to 655.6 +/- 148.69 Kdyn/cm2 [P <0.001] and septal thickness from 10.5 +/- 2.26 to 9.9 +/- 2.06 mm [P <0.05]. Left ventricular [LV] end-systolic and end-diastolic volumes, ejection fraction, shortening, fractional LV posterior wall thickness and LV mass did not change significantly. The degree of AR did not change significantly during the 3-month follow-up period. Captopril in a dose of 50-100 mg daily improves left ventricular afterload in asymptomatic patients with chronic AR, and a longer period of follow up is required to show its effect on LV mass and the severity of aortic regurgitation

Efecto a mediano plazo del enalapril sobre la función y masa ventricular izquierda en insuficiencia aórtica crónica severa / Effect at medium range of enalapril on left ventricular function and mass in severe chronic aortic insufficiency

Se han demostrado efectos beneficiosos de los vasodilatadores arteriales en pacientes con insuficiencia aórtica crónica (IAC). No existen trabajos que estudien el rol de los inhibidores de la ECA en estos pacientes. Con objeto de evaluar el efcto a mediano plazo de enalapril sobre la dilatación y función ventricular izquierda (VI), estudiamos a 20 pacientes con IAC severa y gran dilatación VI (dimensión sistólica ecocardiográfica > de 50 mm). Los pacientes fueron separados en dos grupos: Grupo E (n=10) que recibieron enalapril 20 mg/día por un plazo de seis meses, y grupo C (n=10) que no recibieron la droga. Los pacientes fueron evaluados clínica y ecocardiográficamente al comienzo y 1,3 y 6 meses de ingresados al estudio. Dos pacientes del Grupo E no completaron el estudio, por mala adherencia al tratamiento. Los pacientes del Grupo E presentaron, a los seis meses de tratamiento, una disminución significativa de la dimensión sistólica ecocardiográfica de VI (52,6 ñ 3,1 vs 55,8 ñ 3,2 mm; p<0,05) y de la masa VI(461 ñ 74 vs 527 ñ 73 g; p<0,05), sin cambios significativos en dimensión diastólica y fracción de acortamiento de VI. Los pacientes del Grupo C no presentaron cambios significativos durante el seguimiento. Estos resultados sugieren un efcto beneficioso del enalapril en pacientes con IAC severa y gran dilatación VI, aunque no está bien definido su impacto en la historia natural de estos pacientes