Síndrome de la válvula pulmonar ausente: Evolución, tratamiento y factores asociados a muerte

SummaryA BSENT PULMONARY VALVE SYNDROME .E VALUATION , SURGICAL TREATMENT AND RISK FACTORS From 1991 to 2003 were studied 33 cases withabsent pulmonary valve syndrome (AVPS): 66%were female, with a medium age of 1.5 yearsold 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, onepatient required ballon pulmonary valvuloplas-ty for pulmonary stenosis; another one requiredsurgery for changing the pulmonary prothesisone and five years after the first surgery. The restof the patients did not present important proble-ms. The five year survival was 95.4% in patientsolder than 6 months and 30.1% in younger pa-tients (p = 0.000). As factors associated to mor-tality were the age younger than six months old(p = 0.003) and mechanical ventilation (p =0.001) in our population. We suggest to delaythe surgery in this group of patients because nosurvival were seen with or without the surgery.In older children with symptoms, the surgery alsomust be delayed in order to avoid more inter-ventions for changing the pulmonary prothesis.(Arch Cardiol Mex 2008; 78: 79-86)