[Total hip arthroplasty in Klippel-Trenaunay syndrome. Case report and literature review]. / Artroplastía total de cadera en el síndrome de Klippel-Trenaunay. Reporte de un caso y revisión de la literatura.

INTRODUCTION: Klippel-Tranaunay syndrome is a rare congenital condition characterized by skin angiomas with arteriovenous malformations and hypertrophy of the soft and bone tissues of one extremity. The clinical case presented herein involved total hip replacement due to arthrosis secondary to avascular necrosis of the femoral head, which resulted in the hypervascularity that occurs with this syndrome. A 37 year-old female was diagnosed with grade IV hip arthrosis and Klippel-Trenaunay syndrome. Laboratory and imaging tests were performed to determine the local conditions for the surgical approach to the hip. The preoperative MR angiography identified the arteriovenous malformation tract to determine the limits of the approach. DISCUSSION: The arteriovenous malformations that occur in this syndrome turn the approach into a challenging one due to the potential bleeding, thus extreme prophylactic and technical measures must be applied. There are very few reports in the literature on this treatment.

[Changes in oxygenation indexes with the use of nitric oxide in the postoperative period of congenital heart disease surgery in patients with severe pulmonary hypertension]. / Cambios en los índices de oxigenación con el uso de óxido nítrico en el postoperatorio de corrección de cardiopatías congénitas con hipertensión pulmonar severa.

UNLABELLED: In this clinical study, 7 pediatric patients with severe pulmonary artery hypertension, secondary to congenital heart disease, received inhaled nitric oxide at doses of 20 ppm in the acute postoperative management of congenital heart repair for 3.5 days. Monitoring included oxygenation index, alveoloarterial oxygen difference, pulmonary and systemic pressure, measurements were recorded previous to the NO administration; 1, 6, 12, 24, 36, 48 and 72 hours after treatment with NO, nitrous oxide (NO2) and seric methemoglobin also were monitored. RESULTS: Average age was 3.6 years. The oxygenation index before inhaling NO was 166 +/- 100, 72 hours following inhalation of NO the oxygenation index was 210 +/- 98. The alveolo-arterial baseline oxygen difference was 270 +/- 145; 72 hours after inhaling NO, it was 163 +/- 167. No decreases in pulmonary and systemic arterial pressure during NO inhalation occured. The levels of NO2 and seric methemoglobin were not toxic. CONCLUSIONS: Treatment with inhaled NO after cardiac surgery in children with severe pulmonary artery hypertension secondary to congenital heart disease may be useful to improve oxygenation with no changes in pulmonary and systemic arterial pressure. Inhaled NO at 20 ppm did not cause toxicity.

[Submammary skin incision for longitudinal medial sternotomy]. / Incisión submamaria para esternotomía media longitudinal.

The submammary incision has been postulated as an alternative in median sternotomy approach since 1960. We used this incision in 32 females patients from 1 to 24 years. The diagnoses were atrial septal defects (ASD) 23 patients, ventricular septal defect (VSD) 2 patients, double outlet right ventricle 1 patient, supravalvular aortic stenosis 1 patient, ASD with VSD 3 patients and partial anomalous pulmonary venous connection 1 patient. Nine patients had a complication: cheloid scar 1 patient, seroma 2 patients, wound dehiscence 3 patients, and diminution of sensitivity 3 patients. We conclude, that this type of surgical approach is an excellent alternative for women with good aesthetic and psychological results. Their complications are easy to correct.

[Bidirectional cavopulmonary diversion for tricuspid atresia. Experience in the National Institute of Cardiology]. / Derivación cavopulmonar bidireccional para la atresia tricuspídea. Experiencia en el Instituto Nacional de Cardiología.

Bidirectional cavopulmonary shunt is an alternative palliative procedure for patients with congenital cyanotic heart disease, specially those patients less than "ideal" candidates for a Fontan's procedure. We present our results with this shunt in patients with tricuspid atresia. Twenty patients with tricuspid atresia were operated on with this shunt, with these associated defects: 20 atrial septal defect, 17 ventricular septal defect, 10 pulmonary stenosis, 1 pulmonary atresia and 1 transposition of the great arteries. Sex: 10 males and 10 females; the age was 27 days to 6 years (mean 1.8 years), the weight was 3.2 kg to 24 kg (mean 10.7 kg), the mean pulmonary artery pressure was 11 to 24 mmHg (mean 17 mmHg), pulmonary vascular resistance was 1.5 to 5 UW (mean 3.1 UW). Postoperative oxygen saturation improved 15 to 120%. All patients survived the surgical procedure. Three patients died in the immediate postoperative period, 2 due to a complications in the postoperative period and 1 due to sepsis. There were two late deaths, 1 sudden death after 6 months of the shunt, and 1 due to sepsis after a Fontan's procedure. Four patients presented pleural effusion and 2 pericardial effusion, they resolved well. We have 15 patients alive and well, in functional class I, and minimal cyanosis. We can conclude that this surgical procedure is useful in the management of patients with tricuspid atresia.

[Reconstruction of the normal right ventricle outflow tract in the Ross operation]. / Reconstrucción del tracto de salida normal del ventrículo derecho en la operación de Ross.

Now a day there are many surgical procedures that require intervention on the normal right ventricular outflow tract (RVOT) and its reconstruction. We present the surgical anatomy of the pulmonary root in the normal RVOT and its reconstruction in the Ross operation in 13 patients operated on from February 1992 through February 1994. The surgical excision of the pulmonary valve was done and in order to keep right ventricle-pulmonary artery continuity (RV-PA), autologous pericardium tubes with bovine pericardium valve [done at the Instituto Nacional de Cardiología (INC)], were elaborated during the surgical procedure in all patients. The postoperative period and its clinical status was satisfactory in all cases, without transpulmonary gradient or regurgitation. We conclude that is important to know the surgical anatomy of the pulmonary root in order to avoid irreversible damage. In the other hand, it is worthy to know the different choices to reconstruct RVOT and its postoperative clinical course.

[Complications of the use of the pulmonary flotation catheter in surgery. Prospective study]. / Complicaciones del uso del catéter de flotación pulmonar en cirugía cardiovascular. Estudio prospectivo.

UNLABELLED: We studied 120 insertions of the Swan-Ganz catheter in 119 patients who needed hemodynamic monitorization for cardiovascular surgery. All catheter insertions were carried out soon after the anesthetic induction employing the modified Seldinger technique through the internal jugular vein, taking as reference the intracavitary pressure waves to get the pulmonary artery wedge position. The time of insertion, time of use, complications of puncture, difficulty and complications of insertion, thoracic and pulmonary complications, and loop or knot in the catheter were examined. RESULTS: multiple punctures in 15 cases (12.5%), 8 carotid artery punctures (6.6%), 14 hematomas (11.6%), difficulty for pass through the right ventricle in 8 cases (6.6%) and pulmonary artery in 32 (26.6%) impossibility to get the pulmonary wedge position in 13 cases (10.8%), loop of catheter in 3 (2.5%), ectopic atrial beats in 30 cases (25%), atrial tachycardia in 4 (3.3%), ectopic ventricular beats in 73 (60.8%). General arrhythmia incidence was 67.5%, none serious. All complications were minor and not life-threatening. Only in two cases (1.6%) the complications were major, both pulmonary infarction, but were life-threatening to patients; in 20 patients (16.6%) the outcome was without complications. There were no deaths for the catheter use. The results of this study showed that complications caused by the pulmonary artery flotation catheter were smaller than the benefit obtained in the management of these patients.