Modified Senning Procedure for Treatment of Transposition of the Great Arteries with Crisscross Heart.

CLINICAL DATA: A nine-month-old female infant diagnosed with transposition of the great arteries with symptoms of heart failure associated with cyanosis and difficulty in gaining weight was referred to our center with late diagnosis (at nine months of age). CHEST RADIOGRAPHY: Cardiomegaly; attenuated peripheral vascular markings.Electrocardiography: Sinus rhythm with biventricular overload and aberrantly conducted supraventricular extra systoles. ECHOCARDIOGRAPHY: Wide atrial septal defect, ventricular axis torsion with concordant atrioventricular connection and discordant ventriculoarterial connection. COMPUTED TOMOGRAPHY ANGIOGRAPHY: Concordant atrioventricular connection, right ventricle positioned superiorly and left ventricle positioned inferiorly; discordant ventriculoarterial connection with right ventricle connected to the aorta and left ventricle connected to pulmonary artery. DIAGNOSIS: Crisscross heart is a rare congenital heart defect, accounting for 0.1% of congenital heart diseases. It consists of the 90º rotation of ventricles' axis in relation to their normal position; therefore, ventricles are positioned in the superior-inferior direction rather than anterior-posterior. Most cases have associated cardiac anomalies, and in this case, it is associated with transposition of the great arteries. The complexity and rarity of its occurrence make diagnosis and surgical treatment challenging. OPERATION: Modified Senning procedure using the pericardial sac in the construction of a tunnel from pulmonary veins to the right atrium. Cardiopulmonary bypass time of 147 minutes with nine minutes of total circulatory arrest.

Impact of pulmonary artery reduction during arterial switch operation: 14 years follow-up.

OBJECTIVES: Pulmonary artery/aorta (PA/Ao) size discrepancy plays an important role in the development of neoaortic root growth and valve regurgitation. Since 2004, we started using PA reduction to manage severe great vessels root mismatch at the time of arterial switch operation. The purpose of this study is to evaluate the impact of this technique in the mid- and long-term follow-up. METHODS: Patients considered to have severe PA/Ao mismatch (>2:1 ratio) underwent resection of a 3- to 4-mm flap of the posterior PA wall. Patients submitted to this technique were followed up with clinical and image examinations. Echocardiographic findings were reviewed, and Z-scores were recorded to evaluate the incidence and progression of neoaortic root dilatation and valve regurgitation. RESULTS: The median (Q1-Q3) follow-up time was 8 years (3-11). Before arterial switch operation, the median (Q1-Q3) Z-score of the PA annulus was 2.90 (2.75-3.75). At the latest follow-up, the median Z-score of the neoaortic annulus was 1.34 (0.95-1.66). The mean difference between the Z-scores of PA annulus and neoaortic annulus was 1.56 (P < 0.0001). The mean value of the sinus of Valsalva was +0.29 ± 1, that of sinotubular junction was +0.71 ± 0.6 and that of ascending Ao was +1.09 ± 0.7. There was no severe dilatation of the neoaortic annulus, neoaortic root or ascending Ao during follow-up. Neoaortic valve regurgitation was none or mild in 93% of patients. CONCLUSIONS: PA reduction proved to be a feasible and low-risk procedure to approach PA/Ao mismatch in arterial switch operation. Mid- and long-term follow-up showed a tendency towards stabilization of the neoaortic root dilatation and satisfactory valve performance after the procedure. Further investigation is required with a larger population and longer-term follow-up.

L-Transposition of the Great Arteries.

OBJECTIVES: To describe the various surgical options, perioperative management strategies, and outcomes for this complex lesion. DATA SOURCE: MEDLINE and PubMed. CONCLUSIONS: L-transposition of the great arteries is a relatively rare defect, comprising less than 1% of all congenital heart defects. There is considerable heterogeneity in clinical presentation depending on the presence of associated defects. Surgery for this rare and complex defect has evolved from "physiologic" repair in which the right ventricle remains in the systemic position to the "anatomic" repair, which restores the left ventricle as the systemic ventricle. There has been a great interest in performing the anatomic repair or "double switch" operation, a rather complex operation that presents unique challenges in the postoperative period.

Transposition of Great Arteries with Intramural Coronary Artery: Experience with a Modified Surgical Technique.

OBJECTIVE: Transposition of the great arteries is a common congenital heart disease. Arterial switch is the gold standard operation for this complex heart disease. Arterial switch operation in the presence of intramural coronary artery is surgically the most demanding even for the most experienced hands. We are presenting our experience with a modified technique for intramural coronary arteries in arterial switch operation. METHODS: This prospective study involves 450 patients undergoing arterial switch operation at our institute from April 2006 to December 2013 (7.6 years). Eighteen patients underwent arterial switch operation with intramural coronary artery. The coronary patterns and technique used are detailed in the text. RESULTS: The overall mortality found in the subgroup of 18 patients having intramural coronary artery was 16% (n=3). Our first patient had an accidental injury to the left coronary artery and died in the operating room. A seven-day old newborn died from intractable ventricular arrhythmia fifteen hours after surgery. Another patient who had multiple ventricular septal defects with type B arch interruption died from residual apical ventricular septal defect and sepsis on the eleventh postoperative day. The remainder of the patients are doing well, showing a median follow-up duration of 1235.34±815.26 days (range 369 - 2730). CONCLUSION: Transposition of the great arteries with intramural coronary artery is demanding in a subset of patients undergoing arterial switch operation. We believe our technique of coronary button dissection in the presence of intramural coronary arteries using coronary shunt is simple and can be a good addition to the surgeons' armamentarium.

Transposition of Great Arteries with Intramural Coronary Artery: Experience with a Modified Surgical Technique

Abstract Objective: Transposition of the great arteries is a common congenital heart disease. Arterial switch is the gold standard operation for this complex heart disease. Arterial switch operation in the presence of intramural coronary artery is surgically the most demanding even for the most experienced hands. We are presenting our experience with a modified technique for intramural coronary arteries in arterial switch operation. Methods: This prospective study involves 450 patients undergoing arterial switch operation at our institute from April 2006 to December 2013 (7.6 years). Eighteen patients underwent arterial switch operation with intramural coronary artery. The coronary patterns and technique used are detailed in the text. Results: The overall mortality found in the subgroup of 18 patients having intramural coronary artery was 16% (n=3). Our first patient had an accidental injury to the left coronary artery and died in the operating room. A seven-day old newborn died from intractable ventricular arrhythmia fifteen hours after surgery. Another patient who had multiple ventricular septal defects with type B arch interruption died from residual apical ventricular septal defect and sepsis on the eleventh postoperative day. The remainder of the patients are doing well, showing a median follow-up duration of 1235.34±815.26 days (range 369 - 2730). Conclusion: Transposition of the great arteries with intramural coronary artery is demanding in a subset of patients undergoing arterial switch operation. We believe our technique of coronary button dissection in the presence of intramural coronary arteries using coronary shunt is simple and can be a good addition to the surgeons' armamentarium.

Exercise Capacity Long-Term after Arterial Switch Operation for Transposition of the Great Arteries.

UNLABELLED: Transposition of the great arteries (TGA) is a congenital heart defect successfully corrected through arterial switch operation (ASO). Although this technique had significant impact in improving survival, little is known about the functional capacity of the operated patients long-term after surgery. OBJECTIVE: The aim of this study was to compare the functional capacity of children with TGA long-term after ASO with that of healthy children. DESIGN: Retrospective study. PATIENTS: All patients that performed cardiopulmonary exercise test (CPET) were included in the study. As a control group, healthy children in evaluation for physical activity that performed CPET during the same period were also enrolled. RESULTS: Thirty-one TGA patients (19 male) were compared with 29 age-matched controls (21 male). Maximum oxygen consumption was higher in the control group (45.47 ± 8.05 vs. 40.52 ± 7.19, P = .017), although within normal limits in both groups (above 90% of predicted value). The heart rate behavior during exercise was different in both groups, with a mean chronotropic index significantly lower in the TGA group (63% ± 14 vs. 81% ± 12, P < .001). CONCLUSIONS: Our results showed that exercise capacity long-term after ASO in TGA is well preserved although lower than in healthy children what might be explained by the presence of chronotropic incompetence in the TGA group.

Palliative Senning in the Treatment of Congenital Heart Disease with Severe Pulmonary Hypertension.

BACKGROUND: Transposition of the great arteries (TGA) is the most common cyanotic cardiopathy, with an incidence ranging between 0.2 and 0.4 per 1000 live births. Many patients not treated in the first few months of life may progress with severe pulmonary vascular disease. Treatment of these patients may include palliative surgery to redirect the flow at the atrial level. OBJECTIVE: Report our institutional experience with the palliative Senning procedure in children diagnosed with TGA and double outlet right ventricle with severe pulmonary vascular disease, and to evaluate the early and late clinical progression of the palliative Senning procedure. METHODS: Retrospective study based on the evaluation of medical records in the period of 1991 to 2014. Only patients without an indication for definitive surgical treatment of the cardiopathy due to elevated pulmonary pressure were included. RESULTS: After one year of follow-up there was a mean increase in arterial oxygen saturation from 62.1% to 92.5% and a mean decrease in hematocrit from 49.4% to 36.3%. Lung histological analysis was feasible in 16 patients. In 8 patients, pulmonary biopsy grades 3 and 4 were evidenced. CONCLUSION: The palliative Senning procedure improved arterial oxygen saturation, reduced polycythemia, and provided a better quality of life for patients with TGA with ventricular septal defect, severe pulmonary hypertension, and poor prognosis.

Palliative Senning in the Treatment of Congenital Heart Disease with Severe Pulmonary Hypertension / Senning Paliativo no Tratamento de Cardiopatias Congênitas com Hipertensāo Pulmonar Grave

Background:Transposition of the great arteries (TGA) is the most common cyanotic cardiopathy, with an incidence ranging between 0.2 and 0.4 per 1000 live births. Many patients not treated in the first few months of life may progress with severe pulmonary vascular disease. Treatment of these patients may include palliative surgery to redirect the flow at the atrial level.Objective:Report our institutional experience with the palliative Senning procedure in children diagnosed with TGA and double outlet right ventricle with severe pulmonary vascular disease, and to evaluate the early and late clinical progression of the palliative Senning procedure.Methods:Retrospective study based on the evaluation of medical records in the period of 1991 to 2014. Only patients without an indication for definitive surgical treatment of the cardiopathy due to elevated pulmonary pressure were included.Results:After one year of follow-up there was a mean increase in arterial oxygen saturation from 62.1% to 92.5% and a mean decrease in hematocrit from 49.4% to 36.3%. Lung histological analysis was feasible in 16 patients. In 8 patients, pulmonary biopsy grades 3 and 4 were evidenced.Conclusion:The palliative Senning procedure improved arterial oxygen saturation, reduced polycythemia, and provided a better quality of life for patients with TGA with ventricular septal defect, severe pulmonary hypertension, and poor prognosis.

Fundamento:A transposição das grandes artérias (TGA) é a cardiopatia cianogênica mais comum, com incidência variando de 0,2 a 0,4 por 1000 nascidos vivos. Muitos dos pacientes não tratados nos primeiros meses de vida podem evoluir com doença vascular pulmonar grave, e um método terapêutico para o tratamento desses pacientes pode ser realizado por meio de uma cirurgia paliativa para redirecionamento do fluxo em nível atrial.Objetivo:Relatar a experiência institucional com a cirurgia de Senning paliativo em crianças com diagnóstico de TGA e dupla via de saída do ventrículo direito com doença vascular pulmonar severa. Avaliar, também, a evolução clínica precoce e tardia da operação de Senning paliativo.Métodos:Estudo retrospectivo, baseado na avaliação de prontuários no período de 1991 a 2014. Somente pacientes sem indicação para o tratamento cirúrgico definitivo da cardiopatia em razão de elevadas pressões pulmonares.Resultados:Evidenciou-se após um ano de evolução um aumento médio da saturação arterial de oxigênio de 62,1% para 92,5% e uma redução média do hematócrito de 49,4% para 36,3%. A análise da histologia pulmonar foi possível em 16 pacientes. Em oito pacientes foi evidenciada lesão pulmonar grau 3 e 4.Conclusão:A cirurgia de Senning paliativo melhorou a saturação de oxigênio arterial, reduziu a policitemia e proporcionou uma melhora da qualidade de vida em pacientes com TGA com comunicação interventricular, os quais possuíam hipertensão pulmonar severa com prognóstico fechado.