Estudo hemodinâmico para avaliação da doença valvar / Hemodynamic study for valve disease avaluation

O desenvolvimento das tácnicas do estudo hemodinâmico em cardiologia foi fundamental para a melhor compreensão da fisiopatologia, o aprimoramento diagnóstico e a terapêutica das mais diversas afecções congênitas ou adquiridas. O primeiro relato da utilização de um cateter em um ser humano vivo foi feito por Forssman em 1929. Ele tinha uma formação cirúrgica e dissecou sua própria veia antecubital esquerda e nela introduziu uma sonda uretral de 65cm de comprimento, atingindo a cavidade atrial direita. Este fato foi documentado em uma película radiográfica e, com isto, ficou demonstrada a possibilidade de execução deste método...

Surgical treatment of congenital mitral stenosis: medium-term results

OBJETIVO: Analisar a evolução de pacientes portadores de estenose mitral congênita (EmiC) submetidos a tratamento cirúrgico, buscando estudar os fatores desfavoráveis da plastia valvar e os seus marcadores de durabilidade. MÉTODO: De 1989 a 2002, 23 pacientes foram submetidos a tratamento cirúrgico da EmiC, excluindo aqueles com defeito do septo atrioventricular, e formas univentriculares. A idade mediana foi de 15,5 meses (variação de 2 a 204), e o peso mediano foi de 11 kg (variação 4,5 a 51,6 kg). Anomalias associadas estiveram presentes em 17 (73,9 por cento), complexo de Shone em nove (39,1 por cento) e hipertensão pulmonar em 14 (60,9 por cento). A estenose mitral era grave em 14 (60,9 por cento) pacientes, e moderada no restante (gradiente transmitral mediano de 16 mmHg, variação 8,5 a 32). A plastia valvar foi realizada em 18 (78,3 por cento) pacientes e a troca em cinco (21,7 por cento). As técnicas de plastia incluiram a papilarotomia (n=10), ressecção de membrana supravalvar (n=9) e comissurotomia (n=8). Doze (52,2 por cento) pacientes requereram procedimentos associados. RESULTADOS: Não houve óbitos precoces ou tardios num seguimento médio foi de 58,5 ñ 46,7 meses (variação 1 a 156 meses). O tempo médio de hospitalização foi de 12,7 ñ 8,2 dias. Não foram encontrados fatores de risco associados a insucesso da plastia valvar. A curva atuarial livre de reoperaçäes em cinco anos foi de 67,1 por cento (IC 95 por cento: 56,8 por cento a 77,4 por cento). O grupo submetido a plastia valvar necessitou de reoperação em oito (44,4 por cento) pacientes, sendo dois precoces e seis tardios, comparados com uma (20 por cento) reoperação no grupo submetido à troca valvar. A presença de hipertensão pulmonar no pré-operatório esteve relacionada significativamente (p<0,005) a maiores taxas de reoperação. Todos os pacientes, exceto dois, encontram-se em classe funcional I da NYHA e o ecocardiograma mostra no máximo insuficiência e/ou estenose mitral leve. CONCLUSåES: As reoperaçäes foram o maior fator de morbidade no seguimento a médio prazo e a hipertensão pulmonar pôde ser considerada o fator adverso de durabilidade da plastia mitral, a qual foi o tratamento de escolha para a estenose mitral congênita.

Supravalvular congenital mitral stenosis.

Congenital supravalvular mitral stenosis is a rare malformation characterized by the presence of a shelf-like fibrous membrane, with 1 or 2 small orifices, covering and obstructing the mitral valve. The membrane is positioned closely to the mitral valve (and sometimes it is attached to it); therefore, a preoperative diagnosis is inevitably difficult, even with the use of biplane echocardiography. Two patients with supravalvular mitral stenosis aged 3 years and 3 months are described. In 1 patient, a preoperative diagnosis was made, and both successfully underwent correction.

[Congenital mitral stenosis. Experience in 1991-2001]. / Estenosis mitral congénita. Experiencia 1991-2001.

OBJECTIVE: To describe ten years of experience with congenital mitral stenosis. METHOD: All cases with congenital mitral stenosis from January 1991 to 2001 were analized. RESULTS: 16 patients with congenital mitral stenosis were found, mean age 3 +/- 3.08 years. TYPES OF OBSTRUCTIONS: Mitral commisures combined with tendinous cords fusion (45%), parachute mitral valve 37%, supravalvar mitral ring in one, fusion between papillary muscle and leaflets in one, and double mitral orifice in one. Hemodynamic (in mmHg) findings: wedge pressure 18.4 +/- 4, pulmonary artery systolic 61 +/- 21, diastolic 34 +/- 15, mean 47 +/- 18 mmHg, mean transvalvar gradient (MTG) 12.9 +/- 7.3, PRU 5.5 +/- 3.8. Four patients received medical treatment. Surgical results: Six patients underwent mitral repair, and four were subjected to mitral valve replacement (two with previous mitral repair). Global mortality was 2 (12.5%). Follow-up was 3.5 +/- 3 years. There was a significant difference when the initial MTG 22.6 +/- 11.5 mmHg before surgical repair was compared with the final MTG 4.75 +/- 1.5 (P < 0.05), also when initial MTG 12 +/- 2.3 before mitral valve replacement was compared with the final MTG 6 +/- 1.15 mmHg (P < 0.05). CONCLUSION: Most patients are candidates for mitral surgical repair, however at mid term one third will require mitral valve replacement.

Estenosis mitral congénita: experiencia 1991-2001 / Congenital mitral stenosis 1991-2001 experience

Objetivo: Describir la experiencia en estenosis mitral congénita en 10 años. Material y métodos: Se revisaron los casos de enero 1991-2001. Los sobrevivientes fueron evaluados mediante ecocardiografía. Resultados: Fueron 16 enfermos con media de 3 ñ 3.08 (0 a 10 años). Tipos de obstrucciones: fusión de comisuras y cuerdas tendinosas (45 por ciento), válvula mitral en paracaídas 37 por ciento, anillo supravalvular mitral uno, fusión directa de los músculos papilares a las valvas uno y doble orificio mitral otro. Datos hemodinámicos (en mmHg): presión en cuña 18.4 ñ 4, arteria pulmonar sistólica 61 ñ 21, diastólica 34 ñ 15 y media 47 ñ 18, gradiente transmitral (GTM) 12.9 ñ 7.3 y URP 5.5 ñ 3.8. Cuatro enfermos recibieron tratamiento médico. Resultados quirúrgicos. Reparación mitral en 6 enfermos. Implante de prótesis mitral en 4 enfermos (2 con reparación previa). La mortalidad global fue de 2 (12.5 por ciento). El seguimiento global fue de 3.5 ñ 3 años. Cuando se comparó el GTM antes, 22.6 ñ 11.5 y después, 4.75 ñ 1.5 de reparación mitral o implantación de prótesis (12 ñ 2.3 vs 6 ñ 1.15), la diferencia fue significativa P < 0.05. Conclusiones: La mayoría de los enfermos son susceptibles de reparación mitral. A mediano plazo un tercio de éstos requerirá prótesis.

Unsupported valvuloplasty in children with congenital mitral valve anomalies. Late clinical results.

OBJECTIVE: To analyze late clinical evolution after surgical treatment of children, with reparative and reconstructive techniques without annular support. METHODS: We evaluated 21 patients operated upon between 1975 and 1998. Age 4.67+/-3.44 years; 47.6% girls; mitral insufficiency 57.1% (12 cases), stenosis 28.6% (6 cases), and double lesion 14.3% (3 cases). The perfusion 43.10+/-9.50 min, and ischemia time were 29.40+/-10.50 min. The average clinical follow-up in mitral insufficiency was 41.52+/-53.61 months. In the stenosis group (4 patients) was 46.39+/-32.02 months, and in the double lesion group (3 patients), 39.41+/-37.5 months. The echocardiographic follow-up was in mitral insufficiency 37.17+/-39.51 months, stenosis 42.61+/-30.59 months, and in the double lesion 39.41+/-37.51 months. RESULTS: Operative mortality was 9.5% (2 cases). No late deaths occurred. In the group with mitral insufficiency, 10 (83.3%) patients were asymptomatic (p=0.04). The majority with mild reflux (p=0.002). In the follow-up of the stenosis group, all were in functional class I (NYHA); and the mean transvalve gradient varied between 8 and 12 mmHg, average of 10.7 mmHg. In the double lesion group, 1 patient was reoperated at 43 months. No endocarditis or thromboembolism were reported. CONCLUSION: Mitral stenosis repair has worse late results, related to the valve abnormalities and associated lesions. The correction of mitral insufficiency without annular support showed good long-term results.

Valvoplastia sem suporte em crianças com anomalias congênitas da valva mitral: resultados clínicos tardios / Unsupported valvuloplasty in children with congenital mitral valve anomalies: late clinical results

OBJECTIVE: To analyze late clinical evolution after surgical treatment of children, with reparative and reconstructive techniques without annular support. METHODS: We evaluated 21 patients operated upon between 1975 and 1998. Age 4.67 + or - 3.44 years; 47.6 percent girls; mitral insufficiency 57.1 percent (12 cases), stenosis 28.6 percent (6 cases), and double lesion 14.3 percent (3 cases). The perfusion 43.10 + or - 9.50min, and ischemia time were 29.40 + or - 10.50min. The average clinical follow-up in mitral insufficiency was 41.52 + or - 53.61 months. In the stenosis group (4 patients) was 46.39 + or - 32.02 months, and in the double lesion group (3 patients), 39.41 + or - 37.5 months. The echocardiographic follow-up was in mitral insufficiency 37.17 + or - 39.51 months, stenosis 42.61 + or - 30.59 months, and in the double lesion 39.41 + or - 37.51 months. RESULTS: Operative mortality was 9.5 percent (2 cases). No late deaths occurred. In the group with mitral insufficiency, 10 (83.3 percent) patients were asymptomatic (p=0.04). The majorit y with mild reflux (p=0.002). In the follow-up of the stenosis group, all were in functional class I (NYHA); and the mean transvalve gradient varied between 8 and 12mmHg, average of 10.7mmHg. In the double lesion group, 1 patient was reoperated at 43 months. No endocarditis or thromboembolism were reported. CONCLUSION: Mitral stenosis repair has worse late results, related to the valve abnormalities and associated lesions. The correction of mitral insufficiency without annular support showed good long-term results

Valve repair in children with congenital mitral lesions: late clinical results.

Mitral valve repair may be performed without ring support with advantages related to results and complications. The objective of this study was to analyze the long-term clinical results following surgical repair and reconstruction without the use of rings in cases of congenital mitral lesions in children less than 12 years of age. Twenty-one patients who had undergone surgery during the period from 1975 to 1998 were evaluated. The mean age was 4.6 +/- 3.4 years. Females represented 47.6% of the total. Mitral regurgitation was present in 57.1% (12 patients), stenosis in 28.6% (6 patients), and the mixed lesion group represented 14.3% (3 patients). Perfusion time was 43.1 +/- 9.5 minutes and ischemic time 29.4 +/- 10.5 minutes. Follow-up time was 41.5 +/- 53.6 months for the regurgitation group, 46.3 +/- 32.0 months for the stenosis group, and 39.41 +/- 37.51 months for the mixed lesion group. Echocardiographical follow-up time was 37.17 +/- 39.51 months for the regurgitation group, 42.61 +/- 30.59 months for the stenosis group, and 39.41 +/- 37.51 months for the mixed lesion group. Operative mortality was 9.5% (two cases). There were no late deaths. In the regurgitation group, 10 patients (83.3%) were asymptomatic (p = 0.004). In the echocardiographical follow-up, most of the patients had minimal regurgitation. In the clinical follow-up of the stenosis group all patients were in functional class I (NYHA). The mean transvalvular gradient measured by echocardiography was from 8 to 12 mmHg with a mean gradient of 10.7 mmHg. In the mixed lesion group there was one reoperation at postoperative month 43. There were no cases of endocarditis or thromboembolism. Mitral valve repair in congenital lesions is associated with good late results. The majority of cases in the regurgitation group remain asymptomatic and do not require reoperation. Rings or annular support are not necessary in such cases. Satisfactory repair is more difficult to achieve in cases of mitral stenosis due to valvular abnormalities and the seriousness of the associated lesions.

[Percutaneous treatment of multiple heart defects]. / Tratamiento por vía percutánea de múltiples defectos cardiacos.

We describe our experience in 6 cases with multiple congenital heart defects treated by percutaneous intervention. Their age ranged from 2.3 to 10 years (mean 6.1), with follow-up from 1 to 84 months (mean 28.8). Two cases had coarctation of the aorta (AC) and persistent ductus arteriosus (PDA). Two patients had pulmonary valve stenosis (PVS) and PDA, one case with aortic stenosis (AE) and PDA and one case with AC, mitral stenosis and subaortic stenosis (Shone's Syndrome). Ductus arteriosus was occluded in all patients with Gianturco coils or Rashkind occluder. The valvular gradient post balloon decreased in cases with AC from 46 to 9 mmHg, with PVS from 110 to 10 mmHg and with AE from 40 to 14 mmHg. In a 8 year old boy with Shone's syndrome, we performed angioplasty of aortic coarctation, mitral valvuloplasty with Inoue catheter. He was referred to surgery for subaortic repair stenosis. All are asymptomatic at follow-up. In conclusion; percutaneous intervention is possible in patients with multiple congenital heart defects.

[Percutaneous mitral commissurotomy with an Inoue catheter in congenital mitral stenosis]. / Comisurotomía mitral percutánea con catéter de Inoue en estenosis mitral congénita.

We describe a 3-years-old boy, with congenital mitral stenosis, who underwent percutaneous transvenous mitral commissurotomy (PTMC) with Inoue balloon. The mitral gradient decreased from 24 to 4 mmHg, the mitral valvular area (MVA) increased from 1.2 to 1.6 cm2 without modification in mitral regurgitation. One year later the recatheterization showed decreased pulmonary pressures, and a 7.5 mmHg mitral valvular gradient without mitral insufficiency. MVA by echo is 1.8 cm2 with mild regurgitation. As far as we know there is no experience with Inoue balloon in children. We conclude that PTMC with Inoue balloon could be an attractive alternative in some patients with congenital mitral stenosis.

Válvula mitral en "paracaídas": informe de un caso / Parachute mitral valve: Clinic case

Se informa de un lactante que después de haber tenido un periodo neonatal normal presentó una pobre ganancia de peso, fatiga al ser alimentado, infecciones de repetición. A la radiografía del tórax mostró cardiomegalia, con flujo pulmonar aumentado; y en el ecocardiograma, dilatación de la aurícula izquierda, con la válvula mitral con pendiente EF disminuida (12 m/seg), con un sólo músculo papilar y flujo diastólico transmitral turbulento, con área valvular estenótica (0.7 cm²). Con estos datos se hizo el diagnóstico de estenosis mitral por válvula mitral en "paracaídas". Fue llevado a cirugía a los 11 meses de edad y, ante la imposibilidad de hacerle la plastía, se le colocó una prótesis mitral mecánica. La evolución ha sido satisfactoria, con tratamiento médico a base de coumadin, dipiridamol y furosemida. Se resaltan las ventajas de la valoración no invasiva y el éxito de la cirugía

Left ventricular apical approach for the surgical treatment of congenital mitral stenosis.

From June 1987 to October 1991, 12 patients with congenital mitral valve stenosis underwent surgical correction. Their ages ranged from 2 to 74 months. Nine patients were less than 22 months of age. Five patients were in New York Heart Association functional class IV, and seven patients were in class III. In nine, parachute-type mitral stenosis was clearly definite. In three, a complex congenital valvular and subvalvular stenosis was found. Associated anomalies were present in every patient; five had undergone previous operations. Correction of the mitral stenosis was done through an apical left ventriculotomy. The removal of the mitral obstruction starts from below; the papillary muscle was split and the chordae were divided or fenestrated. The commissurotomies were performed from the ventricular aspect of the mitral valve. Associated anomalies were corrected simultaneously. The operative mortality rate was zero. There was one late death, which was unrelated to cardiovascular status. The echocardiographic serial postoperative studies (up to 52 months) showed no significant residual mitral stenosis and normal global and regional function of the left ventricle in all but one patient.

Area da valva mitral estenotica em gestantes portadoras de doenca reumatica: correlacao com o prognostico perinatal / Mitral valve stenosis and its correlation with the development of the conception product

Em um estudo prospectivo, 52 pacientes, divididas em 29 gestantes portadoras de estenose da valva mitral 23 gestantes normais sem intercorrencias clinicas foram acompanhadas durante o ciclo gravidico-puerperal por um obstetra e um cardiologista. As pacientes dos dois grupos foram submetidas a exames de ecocardiodopplergrafia em fases distintas de idade gestacional entre a vigesima quarta e vigesima oitava semanas e a trigesima segunda e trigesima sexta semanas, a fim de medir a area da valva mitral e sua correlacao com desenvolvimento do produto conceptual e as provaveis repercussoes perinatais advindas da patologia. Nao foram encontrados criterios numericos de correlacao entre tamanho da area mitral e os resultados perinatais. Os resultados perinatais do grupo patologico foram considerados bons, nao se registrando nenhuma morte perinatal ou materna. No entanto observamos a presenca de crescimento intra-uterino (31,03 por cento) quando comparado ao grupo controle (8,70 por cento). Permaneceu a indicacao de cirurgia cardiaca por interesse materno.

A ventriculotomia apical esquerda para tratamento cirúrgico da estenose mitral congênita / Apical left ventriculotomy for the surgical treatment of congenital mitral stenosis

Entre junho de 1987 e outubro de 1990, nove pacientes consecutivos, portadores de estenose mitral congênita (EMC) foram submetidos a correçao cirúrgica. Sete tinham valva mitral em paraquedas e dois, outras formas complexas de estenoses. Em todos, a via de abordagem foi a ventriculotomia apical esquerda, sendo dividido, primeiramente, o músculo papilar; depois, as cordas e, finalmente, as cúspides. As lesoes associadas foram corrigidas prévia ou simultaneamente. Todos os pacientes tiveram boa evoluçao imediata. Houve um óbito tardio nao relacionado. O estudo ecocardiográfico seriado pós-operatório mostrou adequada funçao ventricular esquerda. Conclui-se que esta via é de escolha para tratar lesoes estenóticas congênitas complexas da valva mitral.

[Mitral valve disease in infants. Anatomical and functional evaluation by echocardiography]. / Valvulopatía mitral en el lactante. Evaluación anatómica y funcional por ecocardiografía.

To describe the incidence of mitral valve abnormalities among infants aged 0 to 24 months, their anatomic features, site of lesion, severity and associated heart defects, the records of 3,583 such patients submitted for bi-dimensional ultrasound and pulsed Doppler cardiac studies by presumptive heart disease were analyzed. Mitral valve abnormalities were thus detected in 88 of these cases, and were also documented by clinical examination (n: 88), heart catheterization and angiocardiography (n: 11), surgery (n: 17) and necropsy (n: 2). Seventy five cases had additional heart disease, most commonly aortic stenosis (27%), aortic coarctaction (26%) and ventricular septal defects (43%). Papillary muscle deformities at the subvalvar mitral apparatus were considered to be the most frequent mechanism for congenital mitral stenosis (100%), and annulus dilatation was the most frequent cause of mitral insufficiency (49%). Relative incidences of mitral valve stenosis and insufficiency among the whole studied sample were 0.5% and 1.48% respectively. There was a rough correlation between Doppler transmital gradient or regurgitation jet area and the corresponding mitral valve abnormality. This kind of noninvasively obtained data about mitral architecture and function seems to be a very useful and sensitive guide to define and manage this patients.

Estenosis mitral congénita: comunicación de un caso / Congenital mitral stenosis: report of a case

Se presenta a una paciente de 4 años y 6 meses de edad, con estenosis mitral congénita severa aislada, a quien se le implantó una válvula mecánica Bjork Shiley No. 25. Esta ha sido la menor paciente a quien se le ha practicado este proceder en nuestro país según información disponible. Se exponen los resultados clínicos, los métodos de investigación cruentos e incruentos y la evolución posoperatoria.