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Treatment of the aneurysms comprising the aortic arch is challenging. Surgical reconstruction usually requires aortic cross-clamping, cardiac arrest, and even deep hypothermia for a bloodless field. In this report, we present our surgical technique providing normothermic ascending aorta, aortic arch, and proximal descending aorta replacement with selective cannulation and perfusion of the whole body.
Isolated left subclavian artery from the pulmonary trunk is a rare congenital cardiovascular malformation. In this report, we present the images of ascending aortic aneurysm and left subclavian artery originating from the pulmonary artery in a 4-year-old girl in addition to her congenital cardiac pathology.
Congenital absence of unilateral pulmonary artery, either as a primary defect or in combination with other congenital cardiovascular malformations, is very rare. Double outlet right ventricle pathology in combination with unilateral absence of pulmonary artery has only been reported once in the literature. In this report, we present our experience with double outlet right ventricle with unilateral absence of left pulmonary artery in a 3-year-old female patient who underwent a palliative procedure and was scheduled for correction.
Tetralogy of Fallot is a common congenital cardiac malformation. A rare subgroup includes unilateral absence of the pulmonary artery, either the left or the right main branch. The literature lacks an established treatment for these cases, and surgical options carry certain mortality and morbidity.
Patients and methods
There were five patients who had single pulmonary artery and received surgical treatment among the 126 patients with the diagnosis of Tetralogy of Fallot, who were admitted to our institution between July, 2010 and November, 2011. All the patients were male. Ages ranged between 12 months and 8 years. The mean body mass index was 17.1 plus or minus 3.4 kilograms per square metre. Pulmonary artery Nakata index, Nakata index Z-score, and the McGoon index were used for the quantitative assessment of the pulmonary artery and to determine the surgical strategy.
Urgent modified Blalock–Taussig shunt operations were performed in two patients with very low oxygen saturation and haemodynamic instability. These patients are scheduled for corrective procedures on an elective basis. There was one patient who received an elective shunt procedure; however, the post-operative course was complicated with the overflow phenomenon and the patient underwent total correction with a check-valved patch used to close the ventricular septal defect. The patient required extracorporeal membrane oxygenator support in the post-operative period. There were two patients who underwent total correction of the pathology uneventfully. Mortality did not occur. Mean durations of hospital stay and follow-up were 14 plus or minus 13.4 days and 184.5 plus or minus 89.3 days, respectively.
Our modest series with Tetralogy of Fallot with unilateral absent pulmonary artery indicates the feasibility of surgical correction in patients with appropriate unilateral pulmonary artery size and palliative procedures when the pulmonary artery size is smaller than that predicted for the age. Multi-centre long-term data of larger series are warranted in order to establish a treatment protocol.
This study aimed to describe the pre-operative and operative findings, as well as the post-operative haemodynamics of patients operated on for hypoplastic left heart. The findings of patients who survived or did not survive were also compared to anticipate the risk factors for mortality.
We retrospectively reviewed the anaesthetic and intensive care records of 11 (seven male and four female) patients who underwent a modified Norwood operation as neonates. There were eight patients who survived, while three did not survive after the operation. Haemodynamics, oxygenation, and medications of patients were recorded for the pre-operative and post-operative conditions for 2 days, and compared between groups of patients who survived and those who did not.
The normalised modified Blalock–Taussig shunt area was 3.28, 0.57 square millimetres per kilogram (mean, standard deviation) for the group of patients who survived and 3.55, 1.4 square millimetres per kilogram for the group of patients who did not survive (p = 0.51). The group of patients who survived had a significantly larger normalised aortic annulus area (3.3, 0.89 square millimetres per kilogram versus 1.68, 0.21 square millimetres per kilogram, p = 0.01), lower median age (5.57 (3–8) days versus 46.67 (4–90) days, p = 0.02), and lower weight (2.95, 0.46 kilograms versus 3.85, 0.56 kilograms, p = 0.03) than the group of patients who did not survive. Furthermore, the group of patients who did not survive had a significantly worse pre-operative condition, lower systemic venous and arterial oxygen saturation, and need for a high dose of drugs in the pre-operative and post-operative periods (p was less than 0.05 for each variable).
The pre-operative, operative, and post-operative findings may be related to mortality early after the modified Norwood operation for hypoplastic left heart syndrome.
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