1/20. Repair of aortic atresia and hypoplastic left heart syndrome without using graft material.We present a modification of the Norwood stage 1 operation, where the neo-aorta was reconstructed without using graft material. After extensive mobilization of the descending aorta the ductus arteriosus was excised and an end-to-end anastomosis was created between the main pulmonary artery and the distal ductal-aortic junction. The proximal ascending aorta was anastomosed side-to-side to the neo-aorta. Finally either a direct side-to-side anastomosis was created or a 3.5-mm Gore-Tex graft was implanted between the innominate artery and the right pulmonary artery. The first three patients had a favourable outcome: echocardiography showed good ventricular function and acceptable saturation (85%) at the follow up.- - - - - - - - - - ranking = 1keywords = atresia (Clic here for more details about this article) |
2/20. Precoronary stenosis after stage I palliation for hypoplastic left heart syndrome.We report a patient with stenosis of the native ascending aorta after palliation of hypoplastic left heart syndrome and aortic atresia. We describe the approach to diagnosis, temporary support with extracorporeal membrane oxygenation, and successful surgical reintervention. Stenosis of the native ascending aorta is an important, potentially reversible cause of acute, early postoperative ventricular dysfunction.- - - - - - - - - - ranking = 0.25keywords = atresia (Clic here for more details about this article) |
3/20. Hypoplastic left heart in a female infant with partial trisomy 4q due to de novo 4;21 translocation.We present a female infant with mild dysmorphic features and congenital heart defect: hypoplastic left heart with aortic atresia and hypoplastic aortic arch, ventricular septal defect, and a nonrestrictive atrial communication. Chromosome analysis showed an unbalanced translocation that contained additional material from 4q translocated onto 21q. This resulted in partial trisomy 4 and monosomy for the 21q telomeric region. The derivative chromosome was characterized using G-banding, M-FISH, and whole chromosome painting. The karyotype was described as 46,XX,der(21)t(4;21)(q25;q22.3).ish(wcp4 ;wcp21 ). Additional analyses with FISH probes specific for 21q 22.3, 21q22.2, 21q21.1, and 21q11.2 did not indicate any chromosome 21 duplication within the derivative chromosome 21. monosomy for the telomeric portion of 21q was demonstrated using a tel 21q probe (Oncor). The patient underwent stage 1 Norwood procedure to manage her heart defect. Poor feeding and failure to thrive complicated the postsurgical period. The child subsequently underwent funduplication and feeding tube placement, and at 4.5 months of age presented with microcephaly and developmental delay. Hypoplastic left heart was previously reported with increased frequency in relatively common numeric chromosomal aberrations, such as monosomy X, trisomies 21, 18, and 13, and in various structural chromosomal defects. Our report presents new evidence for the co-occurrence of hypoplastic left heart with a duplicated portion of chromosome 4 distal to 4q25. In addition, monosomy for the telomeric region of chromosome 21 may have implications in the phenotype.- - - - - - - - - - ranking = 0.25keywords = atresia (Clic here for more details about this article) |
4/20. Surgical treatment of coronary sinus orifice atresia with hypoplastic left heart syndrome after total cavo-pulmonary connection.Atresia of the coronary sinus orifice is rare. We describe the surgical treatment of coronary orifice atresia in an infant with a persistent left superior vena cava after total cavo-pulmonary connection for hypoplastic left heart syndrome. The diagnosis was made by cardiac catheterization after total cavo-pulmonary connection at 8 months of age. After surgery, cardiac performance deteriorated. At reoperation, the coronary sinus was fenestrated to the left atrium. The patient survived surgical treatment of coronary sinus ostial atresia unroofed to the left atrium, guiding the placement of the fenestration with a probe placed through the open cardiac end of left superior vena cava.- - - - - - - - - - ranking = 1.5keywords = atresia (Clic here for more details about this article) |
5/20. Isolated left ventricular ischemia after the Norwood procedure.Aortic atresia is the most severe variant of hypoplastic left heart syndrome (HLHS), and has been associated with significant mortality after stage I palliation. Coronary artery abnormalities are more prominent in this group of patients, especially in the presence of a patent mitral valve. Herein, we describe a case of isolated left ventricular ischemia after the Norwood procedure in a neonate with hypoplastic left heart syndrome, left ventricular hypertrophy, mitral stenosis, aortic atresia, and anomalous left coronary artery.- - - - - - - - - - ranking = 0.5keywords = atresia (Clic here for more details about this article) |
6/20. Fetal pulmonary venous flow pattern is determined by left atrial pressure: report of two cases of left heart hypoplasia, one with patent and the other with closed interatrial communication.We report two cases of fetal left heart hypoplasia with extremes of size of interatrial communication, i.e. a closed foramen ovale and an atrial septal defect. Doppler examinations of the pulmonary veins showed almost to and fro flow in the fetus with the closed foramen ovale and, in contrast, a normal flow velocity waveform in the fetus with the atrial septal defect, despite mitral atresia. Our observations support the importance of left atrial pressure in the creation of a pulmonary venous flow pattern in the fetus.- - - - - - - - - - ranking = 0.25keywords = atresia (Clic here for more details about this article) |
7/20. prenatal diagnosis of ventriculocoronary arterial fistula in a fetus with hypoplastic left heart syndrome and aortic atresia.In a fetus with a hypoplastic left heart syndrome detected at 26 weeks' gestation, a communication between the left ventricular cavity and the left coronary artery was detected using color Doppler. Bidirectional flow was demonstrated and the ventricle was shown to be filled via the fistula during diastole. After premature birth at 31 weeks and neonatal death, autopsy confirmed the findings. Because the association of a heart defect with ventriculocoronary communications worsens the neonatal prognosis, it should be ruled out in fetuses with hypoplastic left heart syndrome before parents are counseled.- - - - - - - - - - ranking = 1keywords = atresia (Clic here for more details about this article) |
8/20. prenatal diagnosis of partial anomalous pulmonary venous connection by detection of dilatation of superior vena cava in hypoplastic left heart. A case report.A 27-week fetus was given targeted fetal echocardiography due to an abnormal four-chamber view. A complex heart malformation was seen with two atria, a single ventricle, and aortic and mitral atresia. In addition to intracardiac findings, a dilated superior vena cava (SVC) was seen, suggesting a possible abnormal pulmonary venous connection to the SVC. An increased venous flow (up to 90 cm/s) was recorded as well. The course of pregnancy was uneventful and the baby was delivered vaginally at term. Cardiac surgery was planned but the baby died on 4th day of life. The autopsy findings confirmed both intracardiac anomalies as well as partial anomalous venous connection to SVC, with no apparent obstruction. This is the first report which stresses that dilatation of the fetal SVC may be suspicious for partial abnormal pulmonary venous connection during prenatal life.- - - - - - - - - - ranking = 0.25keywords = atresia (Clic here for more details about this article) |
9/20. Concordance for hypoplastic left heart syndrome in a monochorionic twin pregnancy.The risk of structural heart disease is significantly higher in twin pregnancies than in singleton pregnancies, but the concordance rate has been found to be relatively low, even in monochorionic pregnancies. This is the first report of a monochorionic twin pregnancy concordant for hypoplastic left heart syndrome (HLHS), the diagnosis having been made by fetal echocardiography at 15 weeks' gestation. The findings were confirmed at necropsy at 17 weeks' gestation, following termination of pregnancy. Both twins had mitral and aortic atresia, with severely hypoplastic aortic arches. This report adds weight to there being a genetic component to the cause of HLHS in some cases and illustrates how the findings from early fetal echocardiography with postmortem follow up can help to extend the understanding of the aetiology of this condition.- - - - - - - - - - ranking = 0.25keywords = atresia (Clic here for more details about this article) |
10/20. Spinal anaesthesia in a child with hypoplastic left heart syndrome.hypoplastic left heart syndrome (HLHS), a complex congenital heart disease, is the most common lethal cardiac defect in neonates. Its treatment includes cardiac transplantation and/or surgical palliation. Associated extracardiac congenital abnormalities are exceptional. We report the case of a neonate with HLHS and anorectal atresia who required urgent surgical management to relieve intestinal obstruction. The surgery was successfully performed under spinal anaesthesia.- - - - - - - - - - ranking = 0.25keywords = atresia (Clic here for more details about this article) |
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