Surgical repair for aortopulmonary window with interrupted aortic arch in late childhood.

Aortopulmonary window with interrupted aortic arch is rarely reported beyond infancy. Pre-operative assessment and surgical repair are challenging. We report successful surgical repair of aortopulmonary window with interrupted aortic arch in a 6-year-old girl with near-normal pulmonary artery pressure immediately following surgery.

Surgical Repair of Complex Aortopulmonary Window: A Case Study.

Aortopulmonary septal defect, also known as the aortopulmonary window, is a rare congenital macrovascular malformation. This case involves a 9-year-old boy with aortopulmonary septal defect (type I combined with type IV). Before surgery, milrinone and alprostadil were used to counteract high lung pressure. Surgery was performed under cardiopulmonary bypass, following which the pulmonary pressure decreased. The aorta was cut, and the right pulmonary artery opening was connected with the main pulmonary artery septal defect using polyester patch. An internal tunnel was made, and the deformity correction was completed. The child exhibited normal postoperative recovery with no discomfort. A complex aortopulmonary window is a rare condition that can be treated successfully with appropriate preoperative and surgical management.

Surgical repair of complex aortopulmonary window: a case study

Abstract Aortopulmonary septal defect, also known as the aortopulmonary window, is a rare congenital macrovascular malformation. This case involves a 9-year-old boy with aortopulmonary septal defect (type I combined with type IV). Before surgery, milrinone and alprostadil were used to counteract high lung pressure. Surgery was performed under cardiopulmonary bypass, following which the pulmonary pressure decreased. The aorta was cut, and the right pulmonary artery opening was connected with the main pulmonary artery septal defect using polyester patch. An internal tunnel was made, and the deformity correction was completed. The child exhibited normal postoperative recovery with no discomfort. A complex aortopulmonary window is a rare condition that can be treated successfully with appropriate preoperative and surgical management.

Aortopulmonary window with pumonary atresia with ventricular septal defect with D-transposition of great arteries: extremely rare anomaly.

Aortopulmonary window (APW) is rare a congenital heart disease accounting for 0.1%-0.2% of all congenital heart defects. The 35% of the APW has been associated with wide variety of other structural heart diseases such as ventricular septal defect, persistent ductus arteriosus, arch anomalies and coronary artery anomalies. To the best of our knowledge, only six cases of APW with pulmonary atresia with ventricular septal defect has been described in the literature. It resembles the type 1 truncus arteriosus, and differentiation from this condition is important prior to surgical correction. We present a case of 14-year-old girl child; she was diagnosed with APW with pulmonary atresia with ventricular septal defect and D transposition of great arteries with the help of echocardiography, cardiac catheterisation and cardiac CT.

Valved patch closure of aortopulmonary window.

The case of an 8-year-old boy with an aortopulmonary window who underwent unidirectional valved patch closure of the window is described. The advantages of unidirectional valved patch closure in this setting are discussed.

Surgical management of aortopulmonary window: 24 years of experience and lessons learned.

OBJECTIVES: Aortopulmonary window represents 0.2-0.3% of all congenital heart lesions. Progressive pulmonary arterial hypertension and its consequences are more common with this anomaly. The purpose of this study was to share 24 years of surgical experience in managing a spectrum of 55 cases of aortopulmonary window, followed up to 17 years in a single institution. METHODS: This retrospective study was done from November 1991 to November 2015 of 55 patients with aortopulmonary window who underwent successful surgical repair. Age ranged from 5 months to 31 years with 45 children (12 years and younger) and 10 adults (older than 12 years). The male:female ratio was 2.2:1. The mean weight at operation was 14.63 kg (range 3.5-50 kg). An initial diagnosis was obtained from 2D echocardiography, which showed echo dropout in the parasternal short-axis view. Cardiac catheterization and angiography were performed in 54 out of 55 patients. Cardiac catheterization was not done in 1 patient who was 4 months of age. The mean right ventricular systolic pressure (RVSP) was 94 ± 2 mmHg, and the pulmonary artery mean pressure was 68 ± 2 mmHg. The average left to right shunt was 5.2:1, and the pulmonary vascular resistance index in room air was 7.97 ± 0.5 Wood units, whereas after oxygen administration, it declined to 2.0 ± 0.5 Wood units. Four surgical techniques were used based on the size of the communication and the anatomical conditions. RESULTS: There were no early or late deaths. There were no pulmonary hypertensive crises. All patients underwent echocardiography before discharge; none showed a residual shunt. Mild left ventricular dysfunction was seen in 2 patients. This dysfunction regressed with afterload reduction and diuretics on follow-up. All patients were followed up at intervals of 3 months, 1, 5 and 10 years, with the longest follow-up being 17 years. The mean follow-up period was 7 years. At follow-up, all patients were New York Heart Association class I. The mean RVSP on echocardiography was 32 mmHg at 3 months and 30 mmHg at 7 years with no change on further follow-up. Residual pulmonary hypertension was seen in 3 patients: 2 had mild pulmonary hypertension at 8-years follow-up and 1 had moderate hypertension at 3-months follow-up who required sildenafil postoperatively. CONCLUSIONS: Aortopulmonary window is a rare but well identified and surgically correctable anomaly. Operative repair should be offered as soon as the diagnosis is established, regardless of the patient's age. Irreversible pulmonary hypertension with a right to left shunt despite oxygen administration is the only contraindication for surgery. Various surgical techniques can be applied depending on the size of the communication. Associated arch anomalies may require technically challenging approaches and surgical strategies. Early and long-term outcomes after surgical correction are excellent regardless of age or pulmonary vascular resistance.

Absent aortic valve associated with double outlet right ventricle and aortopulmonary window: physiologic implications of a rare malformation in both the fetus and neonate.

Absence of the aortic valve is a rare congenital heart defect that is detectable in the prenatal period. In this condition, functional aortic valve leaflets are absent; in their place are rudimentary, immobile noncoapting plate-like structures at the level of the annulus resulting in severe aortic regurgitation. We report the fetal diagnosis and subsequent postnatal course of an infant with the novel association of absent aortic valve, double outlet right ventricle, and aortopulmonary window. This unique combination of defects resulted in an unusual pattern of circular shunting that produced evidence of fetal heart failure. Shortly after birth, the abnormal physiology led to compromised systemic perfusion, intestinal perforation and subsequent rapid demise of the patient. Abrupt postnatal deterioration typifies the vast majority of the absent aortic valve cases found in the literature. Our patient is unique in that it is the first female reported with absent aortic valve and the first reported with an aortopulmonary window. This report demonstrates that absent aortic valve is a condition that can be diagnosed prenatally by fetal echocardiogram; the highly abnormal physiology places these patients at risk for fetal heart failure. Given the potential for clinical instability and rapid deterioration after birth, such patients should undergo rapid postnatal assessment and immediate surgical intervention when deemed appropriate.

Sildenafil therapy in complex pulmonary atresia with pulmonary arterial hypertension.

BACKGROUND: Complex pulmonary atresia (CPA) i.e. pulmonary atresia with ventricular septal defect and major aorto-pulmonary collaterals (MAPCAs) or Tetralogy of Fallot with MAPCAs frequently have a clinical course complicated by development of pulmonary arterial (PA) hypertension. METHODS: A cross-sectional retrospective review of patients >16 years with CPA or Tetralogy of Fallot with MAPCAs and PA hypertension treated with sildenafil was conducted. Case notes were reviewed for baseline and follow-up (after sildenafil) characteristics. RESULTS: Five patients, 4 female, median age 28 (range 18 to 47) years, were identified. All patients experienced symptomatic improvement: 2 of 4 wheelchair bound patients responded dramatically and walked 345 and 157 m respectively in 6 min following sildenafil therapy. One of the 4 with marked PA arborization abnormalities and severe ventricular dysfunction had initial symptomatic improvement. Another patient improved from walking less 100 m to climbing 2 flights of stairs. Arterial saturations improved in 2 cases from 70 and 60% to 87 and 84% respectively, whilst arterial saturations remained static in 1 case despite embolization of a classical Blalock-Taussig shunt. One patient with PA arborization/diminished PA bed was unable to tolerate sildenafil. CONCLUSIONS: Sildenafil is well tolerated and leads to symptomatic improvement and better saturations in the majority of patients with CPA with PA hypertension when used in isolation or as an adjunct to percutaneous PA angioplasty.

Compared fate of small-diameter Contegras and homografts in the pulmonary position.

OBJECTIVE: This study analyzes whether small-diameter Contegras behave in the same way as small-diameter homografts, when implanted for the first time in pulmonary position. METHODS: Small-diameter conduits include 12 and 14 mm Contegras and 8-14 mm homografts. Graft dysfunction is defined as right ventricular outflow tract obstruction with peak echo-Doppler gradient>40 mmHg, or grade III/IV graft regurgitation. Graft failure is defined as need for conduit replacement or need for catheter or surgical reintervention. Thirty-eight patients who received small Contegras (n=25) and small homografts (n=13) from October 2002 to end December 2006 were studied. The most frequent indication was pulmonary atresia and ventricular septal defect (n=20; 10 associated with major aorto-pulmonary collateral arteries), followed by truncus arteriosus (n=12). Most patients' characteristics were comparable except that recipients of homografts were smaller (p for body area=0.014). Survival, freedom from graft dysfunction, failure and explantation were estimated by the Kaplan-Meier method. The log-rank test was used to compare outcomes. RESULTS: There were three early and four late deaths. No death was graft related. Survival was 80+/-8.2% for patients with Contegras and 77+/-11.7% for those with allografts: p=0.82. Mean follow-up duration is 22+/-16 months. Freedom from dysfunction for Contegras conduits decreased in the first 6 months and stabilized at 58+/-11% from month 14. For homografts it decreased only 1 year after implantation, down to 35+/-19.7% from month 31: p=0.61. Freedom from Contegras failure diminished the first 16 months to level out at 57+/-13%. No homograft failed the first 2 years. With a p-value of 0.14, homografts tended to fail less frequently. Five grafts were explanted. Freedom from explantation was similar (p=0.98): 90+/-6.7% for Contegras and 75+/-21.6% for homografts at year 3. CONCLUSION: In the first 4 years after pulmonary implantation of small-diameter Contegras and homografts, the fate of both conduits was statistically similar, in spite of different behavior. As Contegras is 'off-the-shelf' available, it constitutes a sound alternative to homograft for right ventricular outflow tract reconstruction in neonates and infants.

Clinical results of staged repair with complete unifocalization for pulmonary atresia with ventricular septal defect and major aortopulmonary collateral arteries.

OBJECTIVE: Our treatment strategy for pulmonary atresia with ventricular septal defect (VSD) and major aortopulmonary collateral arteries is a staged repair that comprises the first complete unifocalization (UF) with 'unification' of intrapulmonary arteries and then the definitive repair. The purpose of this study is to evaluate the outcome of our staged repair strategy with complete UF and to determine the results of our current management strategy. METHODS: From 1982 to 2004, 113 consecutive patients were treated with staged repair at our institute. We evaluated the risk of definitive repair failure or death in the 3 years after definitive repair using logistic regression. Furthermore, we compared the early group (patients who underwent UF before December 1995) and the late group (patients who underwent UF after January 1996). RESULTS: The mean follow-up interval was 8.8 years (0.8 months to 23.3 years), and Kaplan-Meier-estimated overall survival rates after first UF were 80.9, 73.8, and 69.9% at 5, 10, and 15 years, respectively. Survival in patients with an absent central pulmonary artery (PA) was significantly lower than in those with a central PA (p<0.05), and the factor that was significantly associated with definitive repair failure or death in the 3 years after definitive repair was central PA morphology (p<0.05). Higher mean PA pressure after UF was detected in patients with hypoplastic central PA, compared with those without hypoplastic PA (30.9 mmHg vs 23.3 mmHg, p<0.05). In the late group, age (in years) at first UF (3.9 vs 8.4, p<0.01), second UF (4.3 vs 9.2, p<0.01), and definitive repair (5.8 vs 9.1, p<0.01) was significantly younger than in early group, and the survival rate after first UF in the late group was 96.2 and 91.3% at 3 and 7 years, respectively. Systolic right ventricular pressure and the pressure ratio between the right and the left ventricles after definitive repair in the late group were significantly lower than in the early group (53.6 mmHg vs 75.0 mmHg, p<0.01; 61.7% vs 75.9%, p<0.05). CONCLUSIONS: Hypoplastic central PA was a significant risk factor in this disease. The overall survival was improved by our current management strategy. Improved RV pressure after definitive repair appears to affect the long-term outcome.

Successful use of a new Amplatzer Vascular plug for percutaneous closure of a large aortopulmonary collateral artery in a pulmonary atresia with ventricular septal defect prior to complete repair.

We report the case of a 14 year-old girl with a pulmonary atresia with VSD and multiple aortopulmonary collaterals who underwent a successful complementary occlusion of a large collateral vessel using an Amplatzer vascular plug after a previously failed attempt of occlusion with a coil. The percutaneous procedure, performed from the femoral artery before the complete surgical repair, provided an immediate closure of the vessel. This new device is safe and effective for the occlusion of aortopulmonary collaterals, specifically if of large dimensions.

Aortopulmonary window with anomalous origin of the right coronary artery from the pulmonary artery: two cases highlighting the importance of complete pre-operative echocardiographic evaluation of the coronary arteries in all conotruncal anomalies.

This report describes two infants with an aortopulmonary window in association with anomalous origin of the right coronary artery from the pulmonary artery. In both cases the diagnosis was made pre-operatively by transthoracic echocardiography, with the initial clue being extensive collateral flow within the myocardium. In each case there was surgical confirmation of the echocardiographic findings. These two cases demonstrate that coronary artery evaluation should be an integral part of every new echocardiographic evaluation, particularly in the setting of conotruncal anomalies.

Transposition of great arteries with aortopulmonary window: an unusual cause of prepared left ventricle at 11 months.

In patients with transposition of great arteries, presence of aortopulmonary window is very uncommon and associated with high morbidity and mortality. This report describes the case of an 11-month-old female patient in which aortopulmonary window was restrictive, and protected the patient from developing pulmonary vascular disease. The patient underwent successful arterial switch and repair of aortopulmonary window.

Defeito do septo atrioventricular forma total / Atrial and ventricular heart septal defect total form

Nesta monografia é abordada a experiência adquirida durante anos de prática cirúrgica no tratamento dos defeitos do septo atrioventricular, sendo apresentada a opinião dos autores sobre a sua divisão em diferentes formas (parcial, intermediária e total). São apresentados inicialmente, alguns fatos históricos sobre a doença, destacando-se nomes e contibuições para o diagnóstico e o tratamento cirúrgico desta entidade tão complexa. O desenvolvimento embriológico do sistema cardiocirculatório é seguido pelo posicionamento com relação à gênese do aparecimento deste defeito cardíaco e pela noção de que as diferentes formas fazem parte de uma única doença em diferentes fases evolutivas...

Janela aortopulmonar: análise clínico-cirúrgica de 18 casos / Aortopulmonary window: clinical and surgical assessment of 18 cases

O objetivo aortopulmonar (JaoP) é uma anomalia congênita incomum cujos achados clínicos dependem do tamanho do defeito e das lesões associadas. Assim, avaliamos a nossa experiência, comparando-a com 296 casos relatados na literatura. Métodos - Estudo retrospectivo de 18 pacientes (idade variando de 13 dias a 31 anos, sendo 13 (72,2 por cento) do sexo feminino), divididos em dois grupos: grupo A (GA): 10 pacientes com defeito isolado e grupo B (GB): 8 pacientes com defeitos associados. Resultados - Insuficiência cardíaca ocorreu em 14 pacientes, principalmente no GA (8 pacientes), e cianose em três, dois no GB (tetralogia de Fallot (TF) e dupla via de saída de ventrículo direito (DVSVD)) e um com hipertensão pulmonar. Em 5 (38,4 por cento) pacientes do GA, foi diagnosticado insuficiência mitral devido ao sopro sistólico e à sobrecarga do VE ao ECG. No GB, os achados clínicos dependiam dos defeitos associados. O diagnóstico pela ecocardiografia foi feito em 11 (61,2 por cento) pacientes, sendo confundido com insuficiência mitral em três, com canal arterial em um e mascarado por defeitos importantes do tipo DVSVD (2 casos) e TF (1 caso). O diagnóstico foi realizado pelo cateterismo em 3 (16,6 por cento) pacientes, pela cirurgia em 3 (16,6 por cento) e pela necropsia em um (5,5 por cento). Foi feita sirurgia em 14 (77,7 por cento) pacientes com um óbito imediato, e boa evolução a longo prazo nos demais. Conclusão - O diagnóstico de JaoP pode ser confundido com outros defeitos. Os achados clínicos aliados ao estudo e ecocardiográfico podem orientar o diagnóstico do defeito, se analisados adequadamente.

Aortopulmonary window. Clinical and surgical assessment of 18 cases.

OBJECTIVE: Aortopulmonary window (APW) is an uncommon congenital malformation. Its clinical presentation is dependent on the size of the defect and on the associated lesions. We evaluated our experience with this anomaly and compared it with 296 cases reported in the literature. METHODS: Retrospective study of 18 patients diagnosed as having APW (age range from 13 days to 31 years, 13 (72.2%) females), divided into two groups: Group A (GA): 10 patients with isolated APW, and Group B (GB): 8 patients with associated lesions. RESULTS: Heart failure occurred in 14 patients, and cyanosis in 3:2 from GB (tetralogy of Fallot--TF, and double outlet right ventricle--DORV), and one from GA with pulmonary hypertension. In 5 patients from GA the diagnosis of mitral regurgitation was made based on a systolic murmur and LV hypertrophy on the EKG. In GB, clinical findings were determined by the associated defect. Diagnosis was established by echocardiography in 11 (61.2%) of the patients. In 3 patients, a wrong diagnosis of mitral regurgitation was made, in 1 a patent ductus arteriosus was diagnosed and in 3 others, the diagnosis of APW was masked by other important associated defects (2 cases of DORV and 1 case of TF). The diagnosis was made by catheterization in 3 (16.6%) patients, by surgery in 3 (16.6%) and by necropsy in 1 (5.5%). Corrective surgery was performed in 14 (77.7%) patients, with one immediate death and good long-term follow-up in the remaining patients. CONCLUSION: APW can be confused with other defects. Clinical findings, associated with an adequate echocardiogram can provide the information for the correct diagnosis.