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1/11. Differential pulmonary flow in hypoplastic left heart syndrome.

    We report a case of hypoplastic left heart syndrome associated with restrictive interatrial communication and partial anomalous pulmonary venous connection via a right lower pulmonary vein draining to the inferior vena cava. We found unequal pulmonary artery pressure and different pulmonary artery structure, with the right pulmonary artery being lower in pressure and more tortuous and dilated in its peripheral branches than the left. This was attributed to the variant degrees of pulmonary venous obstruction. The left pulmonary venous return was severely obstructed by the restrictive interatrial communication, whereas the anomalous right lower pulmonary vein drained into the inferior vena cava, with less obstruction. To the best of our knowledge, there has never before been a report of differential pulmonary flow associated with a partial anomalous pulmonary vein, in a case of hypoplastic left heart syndrome.
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2/11. 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.
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3/11. Restrictive interatrial communication with protein-losing enteropathy and coagulopathy in hypoplastic left heart syndrome after Norwood palliation.

    The Norwood procedure is one option for neonates born with hypoplastic left heart syndrome. We describe a case of an infant with hypoplastic left heart syndrome, palliatively repaired with the Norwood procedure. The infant developed restriction of the interatrial communication, despite atrioseptectomy at the first stage of palliation. Consequently, a protein-losing enteropathy with severe coagulopathy developed which resolved after a repeat atrioseptectomy.
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4/11. 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.
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5/11. 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.
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6/11. ventricular function determination during extracorporeal membrane oxygenation (ECMO) following Norwood operation: a case report.

    extracorporeal membrane oxygenation has been used successfully to support both cardiac and pulmonary function following Stage I Norwood operation. Determination of the return of native cardiac function and pulmonary function can be easily accomplished because of the single ventricle physiology. The pulmonary function can be assessed while on full flow ECMO by isolating the membrane oxygenator gas compartment, allowing evaluation of native pulmonary gas exchange through the modified Blalock-Taussig shunt. cardiac output can be calculated by using the following oxygen delivery equation: Total O2 delivery = ECMO oxygen delivery ventricular oxygen delivery. The ventricular O2 saturation used in the formula for oxygen delivery is same as the mixed venous O2 saturation returning to the ECMO pump because of the large atrial communication following the Norwood operation. A 3.2 kilogram patient was placed on a pediatric ECMO circuit utilizing a heparin-coated centrifugal pump and a microporous membrane oxygenate after failure to wean from bypass because of a low oxygen saturation and poor ventricular function. On day 1 of support, the systemic arterial oxygen saturation was 100% and matched the ECMO arterial saturation. On day 2 of the support, the patient's arterial saturation decreased to 96%, and the ECMO mixed venous saturation was 87%. Using the oxygen delivery formula, the ventricular cardiac output was calculated to be 175 mL/min, with an ECMO flow of 400 mL/min for a total cardiac output of 575 mL/min. The native ventricular contribution was, therefore, 30% of total cardiac output. Calculation of cardiac output would normally require a left ventricular sample in a patient with biventricular physiology. The single ventricle physiology in the post-operative Norwood patient makes this calculation a useful tool for assessing return of ventricular function in these patients.
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7/11. Restrictive interatrial communication in hypoplastic left heart syndrome after modified Fontan repair.

    The occurrence of pulmonary venous obstruction after total cavopulmonary connection with intraatrial lateral tunnel is a rare occurrence. We present two cases of hypoplastic left heart syndrome with restrictive interatrial communication presenting late after this type of modified Fontan repair. This occurred even after complete excision of the atrial septum at the time of Stage 1 Norwood in both cases. A novel approach to this problem of resecting the roof of the coronary sinus was utilized to enlarge the interatrial communication.
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8/11. In utero cardiac fetal surgery: laser atrial septotomy in the treatment of hypoplastic left heart syndrome with intact atrial septum.

    OBJECTIVE: The purpose of this study was to report a novel technique, laser atrial septotomy, for the in utero treatment of hypoplastic left heart syndrome with intact atrial septum. STUDY DESIGN: In utero atrial septotomy by neodymium-YAG laser photofulguration in a fetus with hypoplastic left heart syndrome (HLHS) and intact atrial septum was performed at 30 4/7 weeks of gestation. Percutaneous fetal cardiocentesis was performed to guide a contact (neodymium-YAG) laser fiber into the right atrium with the objective of creating an interatrial communication by photofulguration of the septal tissue. RESULTS: New onset of blood flow from the left to the right atrium was confirmed by color Doppler imaging during the procedure. The neoatrial septal defect remained patent until delivery. A 3400-g neonate was born by spontaneous vaginal delivery at 37 weeks of gestation. A first stage Norwood procedure was performed on the first day of life and surgical correction of an obstructed right pulmonary vein at 3 months. Although pulmonary vascular resistance was normal at cardiac catheterization at 2 months of age, the infant died at 5 months of age from multiple organ failure. An autopsy was declined. CONCLUSION: In utero laser atrial septotomy is feasible. Further experience is necessary to determine the risks and benefits of this technique for the treatment of fetuses hypoplastic left heart syndrome with intact atrial septum.
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9/11. prenatal diagnosis of ventriculocoronary arterial communication in fetuses with hypoplastic left heart syndrome.

    OBJECTIVE: The purpose of this series was to describe the fetal echocardiographic findings in hypoplastic left heart syndrome with aortic atresia and ventriculocoronary arterial communication and implications of these findings. methods: We describe 2 fetuses with hypoplastic left heart syndrome with ventriculocoronary arterial communication diagnosed at 29 and 20 weeks' gestation, respectively. The underlying cardiac anatomy consisted of a hypoplastic left heart and mitral stenosis with aortic atresia. We used color Doppler and pulsed Doppler sonography on the surface of the myocardium to specifically look for coronary arterial flow. RESULTS: By color Doppler sonography, ventriculocoronary arterial communication was shown between the left ventricular cavity and the left coronary artery with characteristic bidirectional flow on pulsed Doppler examination. There was no mitral regurgitation. The left ventricular myocardium was substantially hypertrophied. The first patient underwent surgical Norwood palliation and died after a prolonged postoperative course. The second patient underwent stenting of the arterial duct and bilateral pulmonary artery banding in the catheterization laboratory but died after a few weeks. Implications of ventriculocoronary arterial communication in association with hypoplastic left heart syndrome are discussed. CONCLUSIONS: It is possible to accurately diagnose ventriculocoronary arterial communication on fetal echocardiography. The presence of ventriculocoronary arterial communication is seen exclusively in a subgroup of patients with an aortic atresia and mitral stenosis variant of hypoplastic left heart syndrome. The prognosis is poor in this subgroup of patients.
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10/11. hypoplastic left heart syndrome with intact atrial septum--attempt of an interventional palliation by ductal and interatrial stent implantation.

    In patients with hypoplastic left heart syndrome (HLHS) and intact atrial septum, the blood entering the left atrium cannot egress. emergency treatment interventionally or surgically is mandatory immediately after birth. We describe a patient with HLHS and intact atrial septum who underwent successful transvenous atrial septostomy immediately after birth. When the interatrial communication became restrictive, stent implantation into the arterial duct and into the atrial septum was performed on the 7th day of life. Despite good hemodymanic response, the lung damage was severe and persistent, rendering staged surgical correction impossible. The child died on the 23rd day of life. autopsy showed patent and correct placed stents in the duct and the atrial septum. There was severe dilatation of pulmonary lymphatic and venous vessels, suggestive of long-standing pulmonary venous hypertension. In conclusion, this form of HLHS has a poor prognosis despite early and aggressive interventional treatment.
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