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.

Transpulmonary correction of an isolated aortopulmonary window in an adult.

We describe the operative management of a 65-year-old patient with a congenital type I aortopulmonary window. At surgery, heavy calcifications on the aortic side of the defect, and close proximity with the left coronary ostium, prevented patch repair from the transaortic access. Patch closure through a combined transpulmonary approach was therefore required. This case illustrates unique features of a late-presenting aortopulmonary window.

Primary repair of aortopulmonary window with an interrupted aortic arch in a very low-birth-weight premature neonate.

The aortopulmonary window concomitant with an interrupted aortic arch is a rare occurrence. We successfully performed an emergency one-stage surgical repair of the aortopulmonary window (type 1) concomitant with an interrupted aortic arch (type A) in the case of a very low-birth-weight (1230-g) premature 2-day-old neonate. We describe the diagnosis, surgery, and postoperative course of this rare occurrence.

Mayer-von Rokitansky-Küster-Hauser syndrome in association with a hitherto undescribed variant of the Holt-Oram syndrome with an aorto-pulmonary window.

We report on the association of Mayer-von Rokitansky-Küster-Hauser syndrome (MRKHS) with a unique form of Holt-Oram syndrome (HOS) with an aorto-pulmonary window. A 24-year-old Turkish woman was referred to our hospital because of primary amenorrhoea. Both her vagina and uterus were absent, and the diagnosis of MRKHS was established. Laparoscopic creation of a neovagina by the modified Vecchietti technique was performed. A rare congenital malformation of the heart, namely an aorto-pulmonary window, had required cardiac surgery when the patient was a 6-month-old infant. This cardiac malformation plus associated upper limb anomalies led to the clinical diagnosis of HOS. To the best of our knowledge, this is only the second report in the scientific literature on the concurrence of MRKHS and HOS, and the first published case of HOS with an aorto-pulmonary window as the cardiac malformation.

Revisit on congenital bronchopulmonary vascular malformations: a haphazard branching theory of malinosculations and its clinical classification and implication.

We propose a haphazard branching theory to support the concept of bronchopulmonary malinosculations, and we apply this theory to classify congenital bronchopulmonary vascular malformation (BPVM) based on the anatomical results we have found. Between January 1990 and December 1997, a total of 22 pediatric patients (10 male and 12 female), aged 2 days to 14 years (median, 19.6 months), with congenital BPVM were enrolled in this retrospective study. Study modalities include the clinical features and plain chest films (n = 22) plus at least two of the following: echocardiography (n = 13), barium esophagraphy (n = 2), bronchoscopy (n = 4), contrast bronchography (n = 8), high-resolution direct coronal CT (n = 1) and electron beam or ultrafast CT (n = 1) of the chest, MRI (n = 10), MRA (n = 1), contrast cineangiocardiography (n = 9), surgery (n = 11), or autopsy (n = 2). The salient clinical features were recurrent lung infections in 14 patients, acute respiratory distress in 13, associated cardiovascular malformations in 8, dextroversion in 7, congestive heart failure in 7, dextrocardia in 4, and complex congenital heart diseases in 4. There were abnormal openings (malinosculations) of the pulmonary airway in 20 patients: to an artery in 12, to a vein in 8, and to the lung parenchyma in 9. These 22 patients with congenital BPVM can be classified into bronchial malinosculation (10 cases), arterial malinosculation (2 cases), and bronchoarterial malinosculation (10 cases). Congenital BPVM can be classified in terms of bronchopulmonary malinosculation based upon a haphazard branching theory, in which abnormal communications between two independent systems (primitive foregut system and aortic-pulmonary arch system) occurred coincidentally rather than causally.

[Complex pulmonary vessel alterations in an aorto-pulmonary window in adulthood]. / Komplexe Pulmonalgefässveränderungen bei aortopulmonalem Fenster im Erwachsenenalter.

An aorto-pulmonary window (aorto-pulmonary septal defect) is considered to be a rare congenital cardiac malformation. We report the case of a patient with an unrepaired congenital aorto-pulmonary window 1.4 cm in size which is the largest aorto-pulmonary septal defect yet reported. This patient died at the age of 58 not from the congenital cardiac malformation but from the after-effects of a pulmonary adenocarcinoma diagnosed 2 years previously. Interestingly, the long standing, persistent pulmonary hypertension correlated well with the varying pathomorphological changes seen in the pulmonary vessels. In addition to the complex morphological signs due to the pulmonary hypertension such as the extensive aneurysms found in the left and right main pulmonary arteries (first report of this size), pulmonary vessels with relatively few pathomorphological changes were also found. Hence this first report of such a long survival of a patient with a comparatively large aorto-pulmonary septal defect demonstrates a surprising variety of pathomorphological alterations seen in the pulmonary arteries in the face of excessive pulmonary hypertension. These alterations could be the result of peripheral aneurysmatic formations and thrombembolic stenoses of the lobular or segmental arteries and might provide an explanation for the long survival of this particular congenital cardiac malformation. All these varying findings in the pulmonary arteries emphasise the fact that the pathomorphological findings in lung biopsies might not be representative of the whole lung.

A cross between truncus arteriosus communis and aortopulmonary septal defect: a hitherto undescribed entity.

Case history and necropsy findings of a 5-month-old infant with a unique heart defect with features of truncus arteriosus communis and aortopulmonary defect in combination with severe tricuspid stenosis are presented. There is a wide spectrum of remarkable heart defects between truncus arteriosus communis and aortopulmonary septal defect.

[Surgical treatment of aortopulmonary septal defect].

OBJECTIVE: To study the surgical treatment of aortopulmonary septal defect. METHOD: From January 1983 to December 1996, 5 patients with aortopulmonary septal defect (APSD) (2 male and 3 female) aged from 6 to 34 years (mean 17.4 years) underwent surgical treatment. According to Mori's were classification, 5 patients belonged to type I, 2 type II and 1 type III. The diameters of the defect ranged from 1.0 to 3.0 cm. The operations were performed under moderate hypothermic cardiopulmonary bypass. Pulmonary artery incision was performed, in 3 patients while aortic incision in the other 2. All defects were repaired by Dacron patch. RESULT: There were no operative deaths and postoperative complications. During follow-up of 10 months to 12 years, no late complications and deaths were found. CONCLUSION: The operation should be done early as possible whenever the diagnosis is established. The surgical repair should be taken under cardiopulmonary bypass. To chose an incision on the aorta is more reasonable than on the pulmonary artery. The use the dacron patch can prevent stenosis and recanalization after operation.

Pulmonary artery flap for closure of aortopulmonary window.

A simple technique for closure of aortopulmonary windows using an inverted pulmonary artery flap is described. Long-term results up to 4 years demonstrate normal growth of the great arteries reconstructed with autologous material only.

Magnetic resonance imaging of hearts with atrioventricular valve atresia or double inlet ventricle.

OBJECTIVE: To investigate the effectiveness and limitations of magnetic resonance imaging in defining cardiac anatomy in patients with double inlet ventricle or atrioventricular valve atresia. DESIGN: Magnetic resonance images were reviewed retrospectively without reference to other morphological data. SETTING: A tertiary referral centre for paediatric cardiology. PATIENTS: 18 patients (aged 8 days to 27 years) with a suspected univentricular atrioventricular connection. METHODS: Imaging by a 1.5 T whole body magnetic resonance system with imaging planes adjusted to individual patient anatomy to best define the cardiac morphology. A complete sequential diagnosis obtained from an independent interpretation of the images was compared with the diagnosis obtained from cross sectional echocardiography and angiocardiography. RESULTS: There was substantial accord between the diagnosis from magnetic resonance alone and that from other methods. In the six instances where there was not accord the magnetic resonance diagnosis was considered to be correct in two cases and incorrect in three cases. In the remaining case no consensus could be reached. In eight patients magnetic resonance imaging provided anatomical information additional to that from other methods. The strengths of magnetic resonance were in imaging the pulmonary arteries and their abnormalities and identifying juxtaposed atrial appendanges but there were some deficiencies in identifying Blalock-Taussig shunts. CONCLUSION: Magnetic resonance imaging provided detailed information about all aspects of cardiac morphology in patients with a suspected diagnosis of univentricular atrioventricular connection. Often it provided additional information to echocardiography. Its use in selected patients should give valuable complementary information.

Transesophageal echocardiography in aortopulmonary communications.

The assessment of congenital aortopulmonary communications by transthoracic echocardiography may be suboptimal, particularly postoperatively, due to limited acoustic windows. We performed intraoperative transesophageal echocardiograms in six patients with eight proven systemic-pulmonary communications. Diagnosis included truncus arteriosus (1), aortopulmonary window (1), Waterston anastomosis (3), central Gore-Tex shunt (1), and modified left Blalock-Taussig shunt (2). All communications were accurately demonstrated by transesophageal echocardiography (TEE). The transesophageal technique also provided an assessment of the size of the aortopulmonary communications and the proximal pulmonary arteries. In addition, the gradient across some of the communications could be accurately estimated utilizing the high pulse repetition frequency Doppler. On the other hand, only two of the seven aortopulmonary communications were detected by transthoracic echocardiography. Postoperatively, transesophageal imaging demonstrated unobstructed conduit in five of five patients who underwent conduit repair, as well as intact closure of aortopulmonary communications and concomitant closure of ventricular septal defects.

Cardiovascular cavities cast in silicone rubber as an adjunct to post-mortem examination of the heart.

We used silicone rubber as a material for making post-mortem casts of cavities in 21 human hearts, 16 of them congenitally malformed, 13 after surgical reconstruction (Fontan and Norwood procedures) and 4 after reconstruction had been performed posts mortem for study purposes. The organs were either fresh, or had been perfusion fixed with formalin prior to casting. When suitable silicone rubber and catalyst were used, we found that casts allowed a very clear view of chamber morphology, and, where surgery had been performed, of any vascular constriction or distortion. We made casts of the aortic sinus and coronary arteries in 3 adult hearts, demonstrating the location and extent of indentation due to atheromatous deposits. The resilience and non-adhesiveness of the silicone rubber allowed casts to be extracted without recourse to corrosion. The surrounding tissues, with the exception of trapped trabeculations and valve structures, were then available for conventional pathological examination. We propose that the relatively simple technique has important applications, not practicable using earlier casting materials, for the study of cardiovascular morphology, and for post-mortem evaluation of stenosed or surgically reconstructed vessels.

The surgical management of aortopulmonary window using the anterior sandwich patch closure technique.

Between 1969 and June, 1986, 13 patients with aortopulmonary window were evaluated and operated upon with eleven survivors. All were children with ages ranging from 2 weeks to 2 1/2 years and had a typical aortopulmonary window (Type 1) with a connection between the ascending aorta and main pulmonary artery. Six of them also had associated cardiac anomalies. Operative techniques included both closed and open procedures. Simple ligation was carried out in two, while the remaining 11 patients were operated upon with the aid of extracorporeal circulation. "Sandwich" patch closure was the preferred method which was employed in 7 patients.

Aortopulmonary window in the first year of life: report on 11 surgical cases.

From June, 1975, through April, 1986, 11 consecutive infants with aortopulmonary window were operated on at the Department of Cardiac Surgery, Ospedali Riuniti, Bergamo, Italy. The classic type of aortopulmonary window was present in 6 patients, whereas 4 patients had a ductus type of aortopulmonary window and 1 patient had a double aortopulmonary window, associated transposition of the great arteries, and ventricular septal defect. Operative technique included patch closure of the defect by means of cardiopulmonary bypass using various approaches: transaortic in 3, transpulmonary in 2, and transwindow in 2. The aortopulmonary window was ligated in 3 patients, in 2 of whom an incorrect diagnosis of patent ductus arteriosus was made at cardiac catheterization. Division and suture of the defect through a median sternotomy was performed in the remaining patient, who was moribund on admission. There was a single operative death. All survivors are in New York Heart Association Functional Class I at a mean interval of 6 1/2 years postoperatively.

Anatomy and pathogenesis of aorticopulmonary septal defect.

The pathologic anatomic features and associated cardiac anomalies of 13 patients with aorticopulmonary septal defect (APSD) and of 236 previously reported cases were analyzed. Morphologically, 3 types of APSD were distinguished: a defect with a more or less circular border located about halfway between the arterial valves and the bifurcation of the pulmonary trunk; a similarly located defect in which the border represents a helix; and a usually large defect in which there is no posterior (distal) border. The appearance of these 3 types of defects suggests a different developmental mechanism for each. Approximately half of the cases of APSD are not associated with other cardiovascular anomalies. Of the anomalies that do occur, anomalous origin of a coronary artery from the pulmonary trunk and interruption of the aortic arch (IAA) type A or severe preductal coarctation are seen far more often than expected. An association with DiGeorge syndrome, frequently noted with persistent truncus arteriosus (TA) and IAA type B, was not seen. Certain cardiac anomalies often seen with TA are rarely seen with APSD, and defects often seen with APSD are rarely seen with TA. These observations indicate that APSD and TA are pathogenetically unrelated even though located in the same region of the heart and, unlike TA and IAA type B, APSD is probably not due to a developmental error involving the neural crest.

Aorticopulmonary window in a dog.

An aorticopulmonary window was diagnosed in an 18-month-old dog that had progressive exercise intolerance, hyperpnea, and cyanosis since birth. The dog was euthanatized and necropsied. Microscopic lesions in the lungs were compatible with chronic pulmonary hypertension.

Aorticopulmonary window associated with tetralogy of Fallot. Report of one case and review of the literature.

A case of aorticopulmonary window associated with tetralogy of Fallot is reported with its clinical, hemodynamic, angiocardiographic, surgical, and necropsy findings. The difficulty in diagnosing a tetralogy of Fallot in the presence of a large aorticopulmonary shunt is emphasized. It is also noted that the only means of discovering the infundibular stenosis of the right ventricle is by angiocardiographic study of the right ventricular outflow tract. The difficulty in distinguishing truncus arteriosus from an aorticopulmonary window coexisting with a ventricular septal defect is discussed, and it is pointed out that the only means of differentiating these two anomalies is the angiocardiographic finding of two sigmoid valves. An embryological hypothesis concerning the pathogenesis of this association is also discussed.