ABSTRACT
La tetralogía de Fallot es la cardiopatía congénita cianótica más frecuente del adulto. El síndrome de válvula pulmonar ausente constituye una variante poco frecuente, que representa del 3% al 6% de los pacientes con tetralogía de Fallot. Presentamos el caso de un paciente masculino de 29 años de edad, con tetralogía de Fallot y síndrome de válvula pulmonar ausente, los hallazgos del examen físico y los principales exámenes complementarios; como así también su evolución y una revisión de la literatura.
The tetralogy of Fallot is the most frequent cyanotic congenital heart disease in adult. Absent pulmonary valve syndrome is a rare variant, representing 3% to 6% of patients with tetralogy of Fallot. We present the case of a 29-year-old male patient with tetralogy of Fallot and absent pulmonary valve syndrome, physical examination findings and major complementary examinations; as well as its evolution and a review of the literature.
A tetralogia de Fallot é a doença congênita cianótica mais comum cardíacas em adultos. A síndrome valva pulmonar ausente é uma variante rara, que representa de 3% a 6% dos pacientes com tetralogia de Fallot. Apresentamos o caso de um paciente masculino de 29 anos de idade, com tetralogia de Fallot e síndrome da valva pulmonar ausente, exame físico e os principais estudos complementares; bem como a sua evolução e uma revisão da literatura.
Subject(s)
Humans , Pulmonary Valve , Tetralogy of Fallot , Heart Defects, CongenitalABSTRACT
Objective To investigate the prenatal ultrasonographic features of absent pulmonary valve syndrome APVS Methods The ultrasonographic images follow-up results and the other clinical data of 1 7 fetuses suffering from APVS were retrospectively analyzed According to the difference of the pulmonary artery diameter subjects were divided into pulmonary artery PA dilated group 14 cases and non-dilated group 3 cases The sonographic features of the two groups were analyzed and compared Results All 1 7 fetuses had rudimentary or absent pulmonary valves and stenosis of the pulmonary annulus Moderate or severe regurgitation flowed through pulmonary artery and right ventricular outflow in diastole PA dilated group might be combined with Tetralogy of Fallot double outlet of right ventricle or right ventricular aneurysm there were 85 7% 12 14 with absent ductus arteriosus The forward flow velocity during systole through pulmonary annulus was significantly fast PA non-dilated group could be accompanied by Ebstein's anomaly or tricuspid atresia Ductus arch was always present The forward flow velocity during systole through pulmonary annulus was slow Conclusions The fetal pulmonary artery diameter with APVS can dilate or not Reverse flow during diastolic period which rushes from arteriosus ductus to the right ventricular outflow tract contributes to the prenatal diagnosis of non-dilated PA.
ABSTRACT
Primary repair of the tetralogy of Fallot with absent pulmonary valve syndrome (TOF/APV) is associated with high mortality rates of 17-33%, especially in neonates. Our standard strategy involves a staged repair with a first palliation, performed during the neonatal period, that includes main pulmonary septation with an ePTFE patch, pulmonary arterioplasty for reduction of vascular dilation, and a modified Blalock-Taussig shunt. We performed successful repairs on two neonates with TOF/APV, one symptomatic and the other non-symptomatic, with this strategy. Case 1 : A 7-day-old boy had TOF/APV, with progressively worsening respiratory distress. His left bronchi, superior vena cava and left atrium were compressed by a dilated pulmonary artery, which was repaired by emergency surgery. Decreasing the diameter of the pulmonary artery (PA index from 2,550 to 525) relieved the compressed organs. Case 2 : A 16-day-old boy with TOF/APV with a main pulmonary artery that increased in diameter from 8 to 17 mm in the course of a single day. He was treated in the same fashion as Case 1. At 1 year of age, an intracardiac repair with tricuspid anuuloplasty was performed successfully. This strategy is much safer than a primary repair and is a good choice for neonatal repair of TOF/APV.
ABSTRACT
Objective The aim of this study is to retrospectively analyze surgical management of absent pulmonary valve syndrome(APVS).Methods Between January 2005 and January 2012,totally 11 children with APVS underwent primary surgical correction.There were 7 boys and 4 girls.Age at surgeries range from 1 to 5 years,and wcight from 10.2-17.5 kg,with average (12.3 ± 3.4) kg.Surgical procedures include VSD repair,pulmonary arteries reconstruction and RVOT reconstruction with monocusp valve.5 cases chose Lecompte maneuver as an option to release compression to bronchus,5 cases underwent fibroscopy inspection and airway secretion suction,and 4 cases adapted deep hypothermic circulatory arrest (DHCA)during correction procedures.All survivors are routinely followed-up with echocardiogram.Results All 11 cases survived,2 of them suffered from frequent lungs infections during the first year post surgery.No case exists bronchus or pulmonary arteries compression.Conclusion APVS is a rare congenital heart defect,which may challenge perioperative managements and operations.Ideal surgical correctiou includes RVOT reconstruction,decompression of bilateral bronchus,and airway inspection with fibroscopy.However,compression of intrapulmonary bronchi by abnormally branching pulmonary arteries may expose patients to a relative long time of medication therapy after surgical correction.A large number of patients with long-term follow-up are needed to draw definitive conclusions on this strategy' s effectiveness.
ABSTRACT
Objective To assess the value of prenatal ultrasonography in diagnosing fetal absent pulmonary valve syndrome(APVS). Methods This was a retrospective study of 8 fetuses with absent pulmonary valve syndrome. Fetal echocardiography and segmental analysis were used to determine the atrial situs,atrioventricular connections and ventriculoarterial relationships. Cardiac chamber dimensions,Doppler studies of the atrioventricular valve and semilunar value were recorded, and cardiothoracic ratio were calculated. Results Levocardia,normal situs and atrioventricular connections were present in all cases. All cases showed typical signs:severe dilatation of the pulmonary trunk and it's branches, and concurrent occurrence of pulmonary valve stenosis and regurgitation. In 6/8 there was an associated ventricular septal defect,overriding aorta and absent arterial duct(Fallot-like APVS),while in 2/8 there was an assoiciated intact ventricular septal and patent arterial duct. Extracardiac malformations were detected in 5 cases.Conclusions APVS can be accurately diagnosed by prenatal ultrasonography but we should pay more attention to the ventricular septal and arterial duct,due to the existence of two variants.
ABSTRACT
Tetralogy of Fallot(TOF) with absent pulmonary valve syndrome is a rare pathologic condition which is characterized by absence of pulmonary valve, dilatation of the pal-monary artery involving its left and right branches in addition to the basic pathoanatomies of TOF. In some cases,the enlarged left pulmonary artery may cause compression of the left bronchus. Its complexity deseves some special attention during corrective surgary. Betwleen 1985 and 1998, six patients with this syndrome underwent surgical correction under CPB combined with moderate hypothermic in our hospi-tal. The surgical procedures consisted of resection of the muscular bands present in RVOT causing obstruction and partial excision of the anterior wall of the markedly dilated left and right pulmonary arteries in 2 cases and transannular pericardial patching in 4 cases. One patiant with absence of left pulmonary artery died on the first postoperative day. The other 5 survivors all recovered. Regarding the proper time for surgical treatment on this particular group of patients, it is advisable that in sick children with no respiratory symptoms, corrective surgery be deiyed to the age of 3 or 4 years old. However, when the sick children developed severe respiratory symptoms, corrective surgery should be done as early as possible. In this special situation, a valved transannular patch is preferred.