Outcomes in Neonates with Pulmonary Atresia and Intact Ventricular Septum Underwent Pulmonary Valvulotomy and Valvuloplasty Using a Flexible 2-French Radiofrequency Catheter

PURPOSE: Outcomes in 6 neonates with pulmonary atresia and intact ventricular septum (PAIVS) undergoing radiofrequency pulmonary valvulotomy and valvuloplasty (RPVV) were reported to identify the factors favorable for RPVV as the treatment of choice. MATERIALS AND METHODS: From May 2000 to January 2008, 6 patients with PAIVS were included in this retrospective study. They were aged 1 day to 90 days old. Study modalities included review of recordings of presentations and profiles of chest radiography, electrocardiography, echocardiography, and cardiac catheterization with angiography. Hemodynamic profiles from the echocardiography and the cardiac catheterization were analyzed. RESULTS: Echocardiography showed severe tricuspid regurgitation, membranous atresia of the pulmonary valve, intact ventricular septum, patent ductus arteriosus, and hypoplastic right ventricle in 6 patients. The pulmonary valve annulus were 4.2 to 6.9 mm in diameters, and those of the tricuspid valve were 7.1 to 10.1 mm. Elevated serum level of cardiac enzymes were found in 1 patient with ventriculocoronary communication (VCC). At cardiac catheterization, the ratio of systolic pressure of the right ventricle to that of the left ventricle ranged from 1.43 to 2.33 before RPVV, and from 0.54 to 1.15 after RPVV (p=0.027). The pressure gradients ranged from 76 to 136 mmHg before RPVV, and from 15 to 39 mmHg after RPVV (p=0.028). The echocardiographic gradients ranged from 16 to 32 mmHg within 24 hours after RPVV, and from 15 to 50 mmHg at the follow-ups. CONCLUSION: RPVV can be a treatment of choice for neonates with PAIVS, if there is patent infundibulum, no right-ventricular dependent coronary circulation, and adequate tricuspid valve and pulmonary valve.

Síndrome de la válvula pulmonar ausente. Evolución, tratamiento y factores asociados a muerte / Absent pulmonary valve syndrome. Evaluation, surgical treatment and risk factors

From 1991 to 2003 were studied 33 cases with absent pulmonary valve syndrome (AVPS): 66% were female, with a medium age of 1.5 years old and 11 kg of weight. Twenty seven cases (82%) were associated to Tetralogy of Fallot. Fourteen patients (5 younger than 1 year old) had corrective surgery. After the surgery, one patient required ballon pulmonary valvuloplasty for pulmonary stenosis; another one required surgery for changing the pulmonary prothesis one and five years after the first surgery. The rest of the patients did not present important problems. The five year survival was 95.4% in patients older than 6 months and 30.1% in younger patients (p = 0.000). As factors associated to mortality were the age younger than six months old (p = 0.003) and mechanical ventilation (p = 0.001) in our population. We suggest to delay the surgery in this group of patients because no survival were seen with or without the surgery. In older children with symptoms, the surgery also must be delayed in order to avoid more interventions for changing the pulmonary prothesis.

Abordagem da valva do tronco pulmonar por desvio direito e uso de cânula bicaval: estudo experimental / Approach of the pulmonary valve using right heart bypass and bicaval cannula: experimental study

OBJETIVO: O objetivo deste estudo foi reproduzir a cirurgia de abordagem da valva do tronco pulmonar por desvio direito, avaliando-se uma nova cânula venosa bicaval com balonetes insufláveis para uso por miniacesso. MÉTODOS: Utilizaram-se 15 suínos da raça Large-White, sendo cinco para a padronização técnica das vias de acesso e monitorização hemodinâmica, nove submetidos ao experimento por meio de esternotomia, e um animal excluído da amostra, operado por mínimo acesso ao tórax. O desvio direito foi estabelecido pela drenagem bicaval, sendo a cânula introduzida pela veia jugular interna direita e locada nas veias cavas cranial e caudal. Os parâmetros medidos foram: Pressão Arterial Média (PAM); Freqüência Cardíaca (FC); Saturação O2 (SAT O2); Capnografia (PetCO2) e Temperatura (T). A análise estatística foi feita comparando-se os valores antes e durante o desvio direito. RESULTADOS: No momento pré-desvio direito, obtiveramse os seguintes valores médios: PAM = 90,8 mmHg, FC = 101,6 bat/min, SAT O2 = 93,8 por cento, PetCO2 = 28,4 mmHg, T = 36,1ºC. Durante o desvio direito, obtiveram-se os seguintes valores médios: PAM = 88,1 mmHg, FC = 98,0 bat/min, SAT O2 = 93,1 por cento, PetCO2 = 25,3 mmHg e T = 36,9ºC. Comparandose as médias obtidas entre os dois momentos, verificou-se não haver diferenças significantes para a PAM, FC e SAT O2 e diferenças significantes para a PetCO2 e a T. CONCLUSÃO: A cânula bicaval com balonetes promoveu drenagem eficaz de ambas as veias cavas, permitindo a manutenção dos parâmetros hemodinâmicos durante o desvio direito, sendo possível realizar a abordagem da valva pulmonar.

OBJECTIVE: To reproduce the surgery for correction of pulmonary valve anomalies using right heart bypass and a new bicaval cuffed venous cannula for minimal access surgery. METHODS: Fifteen Large-White pigs were used for this study. The standard technique model was established with the first five pigs, the experiment was done with nine animals by sternotomy, and one was submitted to a minimally invasive procedure, but has been excluded of the sample. Bicaval drainage by a single cannula was obtained through the internal jugular vein. The cuffs were inflated to increase blood flow to the machine. The return was established by putting the arterial cannula into the pulmonary artery trunk. Pre-bypass parameters were measured and compared with the parameters during the right bypass: Median Arterial Tension (MAT); Heart Rate (HR); O2 Saturation (SAT O2); end-tidal CO2 measures (etCO2); Temperature (T). The statistical analysis was done comparing the pre-bypass and during bypass values. RESULTS: Pre-bypass values: MAT: 90.8 mmHg; HR = 101.6 beat/min; O2 SAT = 93.8 percent; PetCO2 = 28.4 mmHg; T = 36.1ºC. During bypass the obtained values were: MAT: 88.1 mmHg; HR = 98.0 beat/min; O2 SAT = 93.1 percent; PetCO2 = 25.3 mmHg; T = 36.9ºC. Comparing the average values between the two abovementioned moments, no significant difference occurred in MAT, HR and O2 SAT; PetCO2 and T presented significant differences. CONCLUSIONS: The bicaval cuffed venous cannula showed efficient drainage of both venae cavae with appropriate hemodynamic parameters during right bypass allowing access to the pulmonary valve.

Diagnóstico pré-natal de agenesia da valva pulmonar com tetralogia de Fallot / Prenatal diagnosis of absent pulmonary valve with tetralogy of Fallot

A agenesia da valva pulmonar é uma entidade rara e definida pela ausência total ou parcial dos folhetos da valva pulmonar, sendo a forma mais comum associada com tetralogia de Fallot. É considerada malformação grave com alta mortalidade perinatal. Neste artigo relatamos o caso de um feto com agenesia da valva pulmonar com tetralogia de Fallot diagnosticado ao ecocardiograma fetal realizado com 22 e 26 semanas de geração e sua evolução. O diagnóstico pré-natal foi confirmado após o nascimento. O recé-nascido progrediu bem inicialmente, porém evoluiu com desconforto respiratório, decorrente de atelectasia pulmonar por compressão do brônquio pela artéria pulmonar dilatada...

[Prevalence of cardiac murmurs in clinically healthy horses of riding schools [Shiraz, Isfahan and Tehran]]

To determine the prevalence of cardiac murmurs in clinically healthy horses of riding schools [Shiraz, Isfahan and Tehran]. Field Study. Five hundred and twenty six horses [475 sporting and 51 non-sporting horses]. Horses were divided into: sporting horses [jumping, race, endurance, training], non-sporting horses [foals, pleasure] and two different age groups:>2 and 2 years old was significantly greater than horses 2 years old can be related with increase in the prevalence of systolic murmurs on the tricuspid valve. This finding may be due to the effect of training and occurrence of eccentric cardiac hypertrophy

Balloon dilatation of pulmonary valve in Tetralogy of Fallot's.

In some children of Tetralogy of Fallot's (TOF) presenting with progressive cyanosis, are palliative Blalock-Taussing (BT) shunt may be required. There are no reports of this modality of management in India, though this has been practiced in the other countries. The author reports an infant with Tetralogy of Fallot's who successfully underwent ballon dilatation of the pulmonary valve. Review of literature shows 332 patients with TOF undergoing pulmonary valve balloon dilatation as an alternative to BT shunt in 12 studies with significant increase in pulmonary artery 'Z' score and low incidence of conversion to shunt. This modality of management should be considered in selected patients to change a palliative surgery to an intervention.

Management of tetralogy of Fallot with absent pulmonary valve: early and mid-term results of a uniform approach.

The operative management of absent pulmonary valve syndrome remains controversial regarding palliative or one-stage correction, the need for pulmonary valve implantation and pulmonary arterioplasty. This retrospective report summarises the experience of a single centre with a view to provide some answers to this controversy. Forty-six consecutive patients including five infants, aged 2 months to 43 years, underwent primary surgical correction during the last 8.5 years. All the patients underwent two-dimensional echocardiography and cardiac catheterisation. Nine patients had mild and 10 moderate pulmonary artery hypertension. Repair consisted of patch closure of the ventricular septal defect and reconstruction of the right ventricular outflow tract. A valve was incorporated in the pulmonary position in 19 patients. Pulmonary arterioplasty was performed only in infants. Overall hospital mortality was 4 out of 46 patients (8.6%). Two out of five infants died accounting for 40 percent mortality. Forty-two survivors were followed up from 4 to 101 months; 40 patients are in functional class I and two in class II. Actuarial survival at 8.5 years was 91 percent. It is concluded that reconstruction of the right ventricular outflow tract with a transannular patch is sufficient in majority of patients. A selective approach to pulmonary valve insertion is recommended in patients with pulmonary hypertension or other anomalies. Pulmonary arterioplasty should be performed as the primary treatment in infants.

Radiofrequency perforation of pulmonary atresia with intact ventricular septum: and initial experience

Pulmonary atresia with intact ventricular septum is a category of cyanotic congenital heart disease which constitutes an emergency in critically-ill newborns. These patients are ductus dependent requiring prostaglandin E[1] infusions until a systemic-to-pulmonary artery shunt is created or they undergo pulmonary valvotomy, either alone or combined with the shunt. Non-surgical [transcatheter] intervention using radiofrequency perforation techniques for the atretic pulmonary valve is now available as an alternative to surgery. We report successful applications of this technique in 3 patients who tolerated the procedure well and had encouraging follow-up results. We conclude that radiofrequency opening of atretic pulmonary valve will have a role in the treatment of pulmonary valve atresia with intact ventricular septum and will be a valuable alternative to surgical pulmonary valvotomy

Tetralogy of Fallot in adults--107 cases.

Clinical and haemodynamic profile of 107 adult patients above the age of 15 years with TOF was analysed. Cardiac catherization and selective cine-angiography were performed in all cases. Infundibular pulmonary stenosis, mal-alignment type of ventricular septal defect, mitral-aortic fibrous continuity and equal systolic pressures in both the ventricles and aorta were considered mandatory for the diagnosis of Tetralogy of Fallot. Aortic regurgitation was seen in 26 cases (24%), tricuspid regurgitation in 22 cases (21%), absent pulmonary valve in 3 cases (3%), branch pulmonary artery stenosis in 9 case (8.4%), major aortopulmonary collaterals in 15 cases (14%), right atrial pressure was more than 10 mmHg in 10 cases (11%) and right ventricular end diastolic pressure more than 9 mmHg in 73 cases (68%). The left ventricular end diastolic pressure was above 13 mmHg in 58 cases (54%).

En torno a la agenesia aislada de la válvula pulmonar en el adulto: ¿cuál es el momento quirúrgico? / Pulmonary valve absence

La agenesia congénita de las valvas pulmonares es una cardiopatía rara que, asociada a otras malformaciones cardíacas, afecta principalmente a los niños y raramente al adulto. La cirugía, electiva o de urgencia, está indicada en el niño sintomático; por otro lado, es discutible la cirugía del adulto asintomático o moderadamente sintomático con agenesia aislada de la válvula pulmonar. Los autores, con base en la experiencia comunicada en la literatura médica, discuten la conducta terapéutica del caso de un joven de 22 años de edad, con agenesia valvular pulmonar y escasa repercusión hemodinámica, que se describe en el presente artículo. A pesar de los escasos reportes sobre agenesia valvular aislada, los autores han llegado a la conclusión de que no es la cirugía una solución satisfactoria. Esta puede ser retardada en tanto el paciente no muestre cambios hemodinámicos importantes de insuficiencia cardíaca o respiratoria que indiquen un tratamiento menos conservador, para cuyo descubrimiento oportuno recomendamos la vigilancia periódica del paciente mediante estudios ecocardiográficos

Atresia funcional da valva pulmonar / Functional atresia of pulmonary valve

Três recém-natos dentro do 1§ dia de vida, portadores de cianose, em decorrência de anomalia de Ebstein de importante repercussäo hemodinâmica, apresentavam aspecto de valva pulmonar imperfurada. Na evoluçäo clínica, verificou-se que a atresia era do tipo funcional, decorrente da baixa pressäo ventricular direita e hipertensäo no território pulmonar

Three newborn children with cyanosis due to Ebstein's anomaly of tricuspid valve with an important hemodynamic repercusssion presented features of imperforation of pulmonary valve. On the follow-up, clinical and laboratorial findings verified that pulmonary atresia was functional resulting from a combination of low right ventricular pressure and high pulmonary vascular resistance

Tetralogy of Fallot with absent pulmonary valve: diagnostic features and management.

Tetralogy of Fallot with absent pulmonary valve is a rare congenital malformation. The diagnostic features and surgical management in ten patients with this malformation have been described. There were five male and five female patients, (age 3 1/2 to 26 years). The common symptoms were recurrent respiratory tract infection and cyanosis. Examination revealed hyperdynamic precordial pulsations, single second heart sound and an early diastolic murmur along the left sternal border in all. Electrocardiograms were indistinguishable from typical tetralogy of Fallot. Chest X-ray revealed cardiomegaly, prominent pulmonary conus and dilated pulmonary arteries in all cases, lung vascularity being variable. Echocardiographic findings were diagnostic and are discussed in detail. Cardiac catheterization and angiocardiography revealed annular stenosis in all, along with aneurysmal dilatation of main and branch pulmonary arteries. Eight patients underwent intracardiac repair. There was no perioperative mortality. All patients have been followed up for 350 months (mean 29 months).

Two dimensional and doppler echocardiographic diagnosis of bicuspid pulmonary valve / Diagnóstico ecodopplercardiográfico de valva pulmonar bicúspide

O diagnóstico de valva pulmonar bicúspide é habitualmente feito à necrópsia, ocorrendo em 7%dos pacientes com estenose pulmonar e associando-se em geral a tetralgia de Fallot ou a comunicaçäo interventricular. É relatado caso e menina de 14 anos com persistência do canal arterial, em que se detectou à ecocardiografia bidimensional valva pulmonar formada por dois folhetos espessados, abrindo-se em cúpula durante a sístole, e projetando-se abaixo do plano de anel valvar na diástole. Complementaçäo do exame com Doppler demonstoru gradiente transvalvar em 21 mmHg. Estes aspectos caractrísticos sugerem a ecocardiografia bidimensional, associada ao Doppler, como método de escolha para o diagnóstico de valva pulmonar bicúspide

The diagnosis of bicuspid pulmonary valve is usually established postmortem, occuring in 7% of patients with pulmonary stenosis and is usually associated to tetralogy of Fallot or ventricular septal defect. The authos report the case of a 14-year-old girl with patent ductus arteriosus in whom 2-D echocardiography disclosed a pulmonary valve formed of two slightly thickened leaflets that openned with the shape of a dome in systole, and moved under the valver ring plane during diastole. A Doppler examination showed a 21 mmHg transvalvar gradient. These characteristic features suggest that 2-D and Doppler echocardiography are the techniques of choice in the diagnosis of bicuspid pulmonary valve.