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1/33. Norwood procedure without circulatory arrest.

    In the Norwood procedure for hypoplastic left heart yndrome, the distal descending thoracic aorta was cannulated just superior to the diaphragm through median sternotomy. In combination with cerebral perfusion through the graft anastomosed to the innominate artery, which was used as a systemic-to-pulmonary shunt later, this technique enabled us to completely avoid circulatory arrest and deep hypothermia throughout the operation.
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keywords = cerebral
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2/33. Atrioventricular canal defect and hypoplastic left heart syndrome as discordant congenital heart defects in twins.

    We report on a twin pair presenting with atrioventricular canal defect (AVCD) with right ventricular dominance in one twin, and classic hypoplastic left heart syndrome (HLHS) in the other. According to the developmental-mechanistic approach, AVCDs belong to the group of extracellular matrix abnormalities, whereas classic HLHS is included among flow lesions. Twin pairs with congenital heart defect (CHD) generally have concordant defects by mechanistic group. The occurrence of AVCD and classic HLHS in twins or siblings has never been reported. Interestingly, hypoplasia of the left ventricle is the anatomic characteristic which unifies the discordant CHDs observed in our twins. The occurrence of CHD in both members of the twin pair implies a strong influence of genetic factors. At present, the genetic basis determining the different cardiac phenotypes observed in our twins is unknown. The report of these peculiar associations may be useful to stimulate further studies and shed light on the etiology of CHDs.
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ranking = 1479.693411047
keywords = ventricle
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3/33. Absence of the aortic valve associated with hypoplastic left-sided heart syndrome.

    In this report we describe the twelfth case in the literature of absence of the aortic valve cusps, associated with hypoplastic left-sided heart syndrome in a neonate. Clinical and hemodynamic conditions in our patient resemble the classical features of this syndrome except for a greater development of the ascending aorta and the left ventricular cavity, due to aortic insufficiency. A patch was unsuccessfully inserted at the aortic annulus to exclude the left ventricle from the circulation. In addition the Norwood operation was performed.
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keywords = ventricle
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4/33. Congenital alveolar capillary dysplasia with misalignment of pulmonary veins associated with hypoplastic left heart syndrome.

    Three full-term infants died in the first month of life with hypoplastic left heart syndrome (HLH) and persistent pulmonary hypertension (PPH). At postmortem examination, they were found to have alveolar capillary dysplasia with misalignment of pulmonary veins (ACD with MPV). The association of HLH syndrome, and ACD with MPV with intestinal malrotation and/or obstruction, is unique. Decreased blood flow in the ascending aorta in fetuses with left outflow tract obstruction might cause vasoconstriction of pulmonary arterioles to maintain cerebral perfusion. vasoconstriction early during embryogenesis might lead to decreased growth and development of alveolar capillaries and pulmonary veins. This results in pulmonary hypertension, and the arterial blood is forced to bypass the deficient capillary bed and can drain only via the anomalous bronchial veins.
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keywords = cerebral
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5/33. 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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ranking = 161.18285123967
keywords = foramen
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6/33. 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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ranking = 1479.693411047
keywords = ventricle
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7/33. Radiofrequency-assisted atrial septoplasty for an intact atrial septum in complex congenital heart disease.

    Septoplasty of the atrial septum was performed with sequential balloon dilation following radiofrequency-assisted perforation of an intact atrial septum in two newborn infants with hypoplastic left heart syndrome and one with double-outlet right ventricle.
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ranking = 1479.693411047
keywords = ventricle
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8/33. 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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ranking = 2959.386822094
keywords = ventricle
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9/33. 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.
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ranking = 1479.693411047
keywords = ventricle
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10/33. Two-dimensional echocardiographic diagnosis of double outlet left ventricle with subaortic ventricular septal defect, pulmonary stenosis, and a hypoplastic left ventricle.

    Double outlet left ventricle (DOLV) is a rare congenital cardiac malformation in which both the pulmonary artery and the aorta arise exclusively or predominantly from the morphologic left ventricle. We describe a case of DOLV with situs solitus, d-loop ventricles, d-malposition of the great arteries (S, D, D), severe pulmonary stenosis, and a subaortic ventricular septal defect in which the left ventricle was also severely hypoplastic. The anatomic features were shown with 2-dimensional echocardiography, and the diagnosis was later confirmed at cardiac catheterization. Many morphologic variations of this malformation have been described but to the our knowledge DOLV with a hypoplastic left ventricle has not been reported before.
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ranking = 19236.014343611
keywords = ventricle
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