Interrupted Aortic Arch

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J Card Surg. 2006 May-Jun;21(3):264-6.
Complete repair of concomitant interrupted aortic arch and partial anomalous pulmonary venous connection.
Chu MW, Sharma K, Tchervenkov CI, Jutras LF.
Division of Pediatric Cardiovascular Surgery, The Montreal Children's Hospital of the McGill University Health Centre, Montreal, Quebec, Canada.

We report a rare case of concomitant presentation of partial anomalous pulmonary venous connection and type A interrupted aortic arch in a 4-week-old, 2.1 kg newborn. She presented in extremis and was found to have a closed ductus arteriosus with the entire left pulmonary venous return obstructed at its connection with the innominate vein. Emergent operative repair was performed in one-stage, consisting of aortic arch reconstruction and anomalous vein translocation. Postoperative recovery was unremarkable and the patient was discharged from hospital 12 days after operation.

Eur J Cardiothorac Surg. 2006 May;29(5):666-73; discussion 673-4. Epub 2006 Apr 12.
Outcomes in patients with interrupted aortic arch and associated anomalies: a 20-year experience.
Brown JW, Ruzmetov M, Okada Y, Vijay P, Rodefeld MD, Turrentine MW.
Section of Cardiothoracic Surgery, James W. Riley Hospital for Children, Indiana University School of Medicine, 545 Barnhill Dr., EH 215, Indianapolis, IN 46202-5123, USA.

OBJECTIVE: The surgical results for the repair of interrupted aortic arch (IAA) have evolved in recent years. We report our results for staged repair of this complex congenital malformation. METHODS: Sixty-five patients (mean age, 16.9+/-41.7 days) were diagnosed with IAA and referred for surgical therapy. The surgical management strategy at our institution between 1982 and 2005 has been one-stage complete repair (n=13) or staged repair (n=52) in selected patients. Non-complex patients (group I, n=51) had a ventricular septal defect (87%), aortopulmonary window (8%), and left ventricular outflow tract obstruction (27%). Group II (n=14) were patients with Taussig-Bing double outlet right ventricle (n=6) or truncus arteriosus (n=8). Method of staged repair of IAA was to transect and turn down the left carotid artery and anastomosis it to the descending aorta (n=41) or graft interposition (n=2) combined with a pulmonary artery (PA) banding followed in a few months by delayed ventricular septal defect (VSD) closure and PA de-banding. RESULTS: There were 5 early and 10 late deaths. The actuarial survival including early mortality was 92% at 1 year, 81% at 5 years, and 76% at 10 and 15 years. There was an 81% 15-year survival for children in group I compared with a 54% for children in group II (p<0.001). Risk factors for increased mortality by univariate analysis were as follows: (1) primary aortic anastomosis (p=0.03), (2) presence of complex anomalies (p=0.05), and (3) initial IAA repair performed before 1994 (p=0.05). Actuarial freedom from any type of aortic reoperation or intervention was 86% at 1 year, 69% at 5 years, and 60% at 10 and 15 years. Univariate and multivariate analyses identified no tested variables as risk factors for reoperation. The majority (86%) was in New York Heart Association (NYHA) class I, and 14% remained in NYHA class II. During the postoperative course there were no neurologic deficits, seizures, and growth disturbances in any patient. CONCLUSION: Staged repair of IAA using a left carotid artery turn down can be safely applied in IAA patients with and without other intracardiac anomalies with good results. Use of the left carotid artery for arch reconstruction did not result in any detectable neurological events or growth disturbances later in life. Associated anomalies played an important role in outcomes. The long-term probability for reoperation and/or reintervention remains high regardless of operative technique.

J Thorac Cardiovasc Surg. 2006 May;131(5):1136-1141.e2.
Surgical management of aortopulmonary window associated with interrupted aortic arch: a Congenital Heart Surgeons Society study.
Konstantinov IE, Karamlou T, Williams WG, Quaegebeur JM, del Nido PJ, Spray TL, Caldarone CA, Blackstone EH, McCrindle BW;
Congenital Heart Surgeons Society.
Hospital for Sick Children, Toronto, Canada.

OBJECTIVE: The objective was to determine outcomes and risk factors of surgical management of patients with aortopulmonary window associated with interrupted aortic arch. METHODS: From 1987 to 1997, 472 neonates with interrupted aortic arch were enrolled prospectively from 33 institutions. Associated aortopulmonary window was present in 20 patients. Competing risk methodology determined the prevalence of reintervention for postrepair pulmonary artery and aortic arch obstruction. RESULTS: Interrupted aortic arch was type A in 17 patients and type B in 3 patients. Aortopulmonary window morphology was type I (n = 10), type II (n = 5), and type III (n = 5). Associated cardiovascular anomalies were common, including atrial septal defect (n = 13) and systemic venous anomalies (n = 3). Overall survival after initial admission was 91%, 86%, and 84% at 1, 5, and 10 years, respectively. Fifteen patients underwent single-stage repair, and 4 patients underwent staged repair. There was an increased prevalence of patch augmentation of the interrupted aortic arch anastomosis in lower-weight infants (2.3 kg vs 3.1 kg, P = .07). Competing risk analysis estimated that 5 years after repair, 51% had initial arch reintervention, 6% had initial pulmonary artery reintervention, and 43% were alive without reintervention. Reintervention for arch obstruction was more likely for those with interrupted aortic arch type B (P = .08) and for those with higher weight at initial repair (P = .003). CONCLUSIONS: Complete correction of aortopulmonary window in the setting of interrupted aortic arch can be performed with low mortality in the neonatal period. Reinterventions for aortic arch obstruction are the most frequent complication after repair, but pulmonary artery stenosis also occurs. Use of patch augmentation may reduce the need for subsequent arch reintervention.

J Thorac Cardiovasc Surg. 2006 Apr;131(4):779-84.
Selective management of the left ventricular outflow tract for repair of interrupted aortic arch with ventricular septal defect: management of left ventricular outflow tract obstruction.
Suzuki T, Ohye RG, Devaney EJ, Ishizaka T, Nathan PN, Goldberg CS, Gomez CA, Bove EL.
Section of Cardiac Surgery, Division of Pediatric Cardiac Surgery, University of Michigan School of Medicine, Ann Arbor, Mich, USA.

OBJECTIVE: Left ventricular outflow tract obstruction remains an early and late complication after repair of interrupted aortic arch and ventricular septal defect. We reviewed our experience with the selective management of the infundibular septum during primary repair to address left ventricular outflow tract obstruction. METHODS: From 1991 through 2001, all 27 patients presenting with interrupted aortic arch/ventricular septal defect and posterior deviation of the infundibular septum were analyzed. Fifteen patients with the smallest subaortic areas underwent myectomy or myotomy of the infundibular septum concomitant with interrupted aortic arch/ventricular septal defect repair. RESULTS: Patients undergoing myectomy-myotomy (Group I) had significantly smaller subaortic diameter indexes (0.83 +/- 0.16 cm/m2) when compared with those who had only interrupted aortic arch/ventricular septal defect repair (group 2: 0.99 +/- 0.13 cm/m2, P = .012). Two hospital deaths occurred in group 1, and 1 occurred in group 2. No late deaths occurred. No patient in group 2 required reoperation. Six group 1 patients required 9 reoperations for left ventricular outflow tract obstruction. Five patients underwent resection of a new subaortic membrane. Only 1 patient had recurrent muscular left ventricular outflow tract obstruction. Three patients required a second reoperation, primarily related to aortic valve stenosis. CONCLUSIONS: Interrupted aortic arch/ventricular septal defect with posterior malalignment of the infundibular septum can be repaired with low mortality in the neonatal period. Tailored to the degree of subaortic narrowing, resection or incision of the infundibular septum at the time of primary repair was very effective in preventing or prolonging the interval to recurrent left ventricular outflow tract obstruction compared with the published data. However, reoperation for left ventricular outflow tract obstruction, often related to the development of a new and discrete subaortic membrane or valvar stenosis, is still required in a subset of patients.

Ann Thorac Surg. 2006 Jan;81(1):214-22.
Truncus arteriosus associated with interrupted aortic arch in 50 neonates: a Congenital Heart Surgeons Society study.
Konstantinov IE, Karamlou T, Blackstone EH, Mosca RS, Lofland GK, Caldarone CA, Williams WG, Mackie AS, McCrindle BW.
The Hospital for Sick Children, Toronto, Ontario, Canada.

BACKGROUND: Patients with both interrupted aortic arch (IAA) and truncus arteriosus (TA) have worse outcomes than those with either lesion in isolation. We determined outcomes and associated factors in this rare group. METHODS: From 1987 to 1997, 50 (11%) of 472 neonates with IAA were identified with TA. Site of aortic arch interruption was distal to the left subclavian artery in 16% and between the left common carotid and subclavian artery in 84%. From the common arterial trunk, the pulmonary arteries arose from a main pulmonary trunk in 46%, common orifice in 22%, and separate orifices in 32%. At presentation, truncal valve stenosis was present in 12% and regurgitation in 22%. RESULTS: There were 34 deaths, with a single early hazard phase. Overall survival from admission was 44%, 39%, and 31% at 6 months, 1 year, and 10 years, respectively. One patient had primary cardiac transplantation and 4 died without any intervention. The IAA repair alone was performed in 7 patients, with single stage repair of both IAA and TA in 38 patients. Associated factors for overall time-related death include female gender (p < 0.001), type III TA (p < 0.001) and one institution (low-risk; p < 0.001). Results improved somewhat over time (p < 0.001). At 5 years after IAA repair only 28% were alive without arch repair intervention, and at 5 years after TA repair only 18% were alive without conduit reoperation. CONCLUSIONS: The combination of IAA and TA carries high early mortality, with high risk of reinterventions in survivors. One stage repair of both TA and IAA is the optimal management.

Dtsch Med Wochenschr. 2005 Dec 16;130(50):2893-6.
Primary diagnosis of an interrupted aortic arch in a 65-year old woman with hypertension
Maier JM, Scheffold N, Cyran J.
Medizinische Klinik I, SLK-Klinikum Heilbronn.

HISTORY AND PHYSICAL EXAMINATION: A 65-year-old woman presented in a hypertensive crisis and with angina pectoris. She had a history of hypertension for several years and medication included five different antihypertensive drugs. On physical examination a faint systolic murmur was heard. Weak femoral pulses were felt, but not the arterial pulses distal to the groin. INVESTIGATIONS: An attempt to perform coronary arteriography failed because it was impossible to pass the catheter across the aortic arch. MR-angiography and cardiac catheterization via the brachial artery confirmed the diagnosis of a complete interruption of the aortic arch distal to the left subclavian artery and showed distinct collateral circulation predominantly via the internal mammary arteries. DIAGNOSIS AND THERAPY: Because of the age of the patient we assumed that the pathogenesis of this interruption of the aortic arch probably was progression and finally occlusion of an aortic coarctation. It was not possible to distinguish the findings from a true congenital atresia, because there was no histological examination. The patient rejected surgery and conservative therapy with frequent monitoring seemed justified, considering the good blood pressure adjustment and the extensive collateral vascularization. CONCLUSION: Patients with complete interruption of the aortic arch very rarely do reach late adult age without previous surgical intervention. Indeed, such a situation is only conceivable when there is a good collateral blood supply and no concomitant shunt defects. There is an indication for operation, but as there are insufficient follow-up data this should be weighed up carefully in elderly patients whose blood pressure is well controlled.

Pediatr Radiol. 2004 Nov;34(11):901-3. Epub 2004 Sep 17.
Interrupted aortic arch in a neonate: multidetector CT diagnosis.
Cinar A, Haliloglu M, Karagoz T, Karcaaltincaba M, Celiker A, Tekinalp G.
Departments of Paediatrics, Hacettepe University Faculty of Medicine, 06100 Ankara, Turkey.

We report the usefulness of multidetector CT angiography (CTA) in the diagnosis of interrupted aorta of a neonate. CTA is useful for evaluating malformations of the aortic arch, particularly in cases that cannot undergo conventional angiography or in which detailed information cannot be

Prenat Diagn. 2003 Sep;23(9):752-7.
22q11 deletions in fetuses with malformations of the outflow tracts or interruption of the aortic arch: impact of additional ultrasound signs. Volpe P, Marasini M, Caruso G, Marzullo A, Buonadonna AL, Arciprete P, Di Paolo S, Volpe G, Gentile M.
Department of Obstetrics and Gynecology, Hospital, Bari, Italy.

OBJECTIVES: One hundred and forty-one consecutive cases of malformations of the outflow tracts or interrupted aortic arch (IAA), detected by fetal echocardiography, underwent detailed anatomy scan, karyotyping and fluorescence in situ hybridization analysis (FISH) to detect the prevalence of 22q11 microdeletion and to evaluate neonatal clinical findings and outcome according to the presence of the genetic defect. Then, we sought to investigate whether some prenatal ultrasound findings could help identify fetuses at higher risk of carrying the 22q11 microdeletion. METHODS: Echocardiography and FISH for the DiGeorge critical region (22q11) were performed in all cases. RESULTS: 22q11 microdeletion was detected in 28 of 141 fetuses (19.8%). Intrauterine growth restriction (IUGR) appeared to be associated with the worst prognosis, being present in 2/2 intrauterine fetal deaths and 5/6 post-natal deaths. IUGR, additional aortic arch anomalies and thymic hypo/aplasia were significantly more frequent in fetuses with 22q11 microdeletion (p=0.011, 0.011 and <0.0001, respectively). Prenatal ultrasound thymus examination, performed on the last 84 fetuses, showed 75% sensitivity and 94% specificity. The combination of 2 predictors, namely, thymus defects and IUGR associated with additional aortic arch anomalies reached more than 90% sensitivity and 100% specificity. CONCLUSIONS: Our study demonstrates that 22q11 microdeletion occurs in 20% of malformations of the outflow tracts and IAA type B, as detected in utero, and that this association is significantly predicted by the presence of associated ultrasound findings: thymic hypo/aplasia, IUGR and additional aortic arch anomalies. The feasibility of a correct prenatal diagnosis should enable clinicians to provide the couple with further informative counselling and to plan adequate post-natal medical interventions. Copyright 2003 John Wiley & Sons, Ltd.

Cardiol Young. 1999 Nov;9(6):562-71.
Echocardiographic assessment of interrupted aortic arch.
Kaulitz R, Jonas RA, van der Velde ME.
Medical School of Hannover, Department of Cardiology, Children's Hospital, Boston, Massachusetts 02115, USA.

BACKGROUND: In patients with interrupted aortic arch echocardiography provides detailed information about the anatomy of the aortic arch and the associated cardiac anomalies. Only a few reports have evaluated the reliability of this non-invasive diagnostic procedure by correlation with angiographic and surgical findings. METHODS: From 1988 through 1993, 45 infants with interrupted arch underwent surgical repair (mean age 13.02 days). Of the patients, 33 had interruption of the arch between the left common carotid and subclavian arteries; 25 patients had a ventricular septal defect, and the remaining 20 had coexisting complex congenital heart defects. Preoperative diagnosis was made exclusively by echocardiography in 25 of the patients. Accuracy of echocardiographic diagnosis was evaluated retrospectively by comparing preoperative studies with angiography and surgical reports. We then investigated whether the morphologic features of the interrupted arch might influence surgical procedure or outcome. RESULTS: Intracardiac anatomy was accurately diagnosed by echocardiography in all cases; in 2 patients angiography provided additional information concerning the morphology of the aortic arch. Operative notes described differences in morphology of the arch in 7 patients, but these did not influence the surgical procedure. Direct anastomosis of the interrupted segments was possible in 38 patients, and 36 patients underwent primary intracardiac repair. Echocardiographic measurements revealed that the diameter of the ascending aorta was related to the number of vessels originating from the proximal aortic arch. The distance between the interrupted segments was significantly different according to the site of interruption, but not between cases with an isolated ventricular septal defect versus those with complex heart disease. It did not influence the method of arch repair, nor was it related to recurrent or residual obstruction. CONCLUSION: Preoperative echocardiography offers accurate and complete diagnosis in the critically ill neonate with interrupted aortic arch and associated intracardiac abnormalities.

Circulation. 1995 Nov 1;92(9 Suppl):II128-31
Interrupted aortic arch. Impact of subaortic stenosis on management and outcome.
Jacobs ML, Chin AJ, Rychik J, Steven JM, Nicolson SC, Norwood WI.
Division of Cardiothoracic Surgery, Children's Hospital of Philadelphia, PA 19104, USA.

Interrupted aortic arch (IAA) is often related developmentally to subaortic obstruction (SAO). When severe, SAO must be addressed in surgical management of IAA. From 1990 to 1993, 25 neonates presented for initial surgical management of IAA complexes. Associated lesions were ventricular septal defect (VSD) with or without atrial septal defect (19 patients), truncus arteriosus (3 patients), tricuspid atresia with transposition of the great arteries (1 patient), aortic atresia with VSD (1 patient), and d-transposition of the great arteries with VSD (1 patient). Overall hospital mortality was 20% (five deaths). One death was related to sepsis and two to sudden hemodynamic decompensation (a 2-kg premature infant after arch repair and VSD closure and a neonate with IAA-truncus arteriosus after arch repair and truncus repair with aortic root replacement). Two deaths were related to low cardiac output in patients with severe subaortic narrowing (< 3 mm by two-dimensional echocardiography), which was not addressed surgically. Of 10 additional patients judged preoperatively to have severe SAO, 1 underwent resection of the infundibular septum together with VSD closure and arch reconstruction, and 9 underwent a modification of Norwood's operation with arch reconstruction and proximal pulmonary artery to aortic anastomosis (7 with systemic to pulmonary artery shunts and 2 with right ventricle to pulmonary artery outflow tract reconstruction). One patient died 2 months after surgery of staphylococcal sepsis. All 9 others were discharged well. Subaortic narrowing is a physiologically important element of IAA complexes. When SAO is severe, satisfactory initial palliation can be achieved by a modification of Norwood's operation.

J Am Coll Cardiol. 1987 Nov;10(5):1072-7.
Accuracy of two-dimensional echocardiography in the diagnosis of aortic arch obstruction.
Nihoyannopoulos P, Karas S, Sapsford RN, Hallidie-Smith K, Foale R.
Department of Medicine, (Clinical Cardiology), Royal Postgraduate Medical School, Hammersmith Hospital, London, England.

To evaluate the predictive accuracy of two-dimensional echocardiography in the diagnosis of aortic arch obstruction, 540 consecutive patients aged 2 days to 15 years (mean 2 months) who underwent subsequent cardiac catheterization and angiography were prospectively studied. At angiography, 51 patients had aortic arch obstruction; of these, 35 had juxtaductal coarctation, 15 isthmic hypoplasia and 1 a type B interrupted aortic arch. The presence of arch obstruction was correctly identified with two-dimensional echocardiography in 45 of 51 patients with this condition (overall sensitivity 88%). Two-dimensional echocardiography clearly defined a juxtaductal coarctation in 33 of 35 patients and isthmic hypoplasia in 13 of 15 patients (sensitivity 94% and 73%, respectively). The form and type of interrupted aortic arch were clearly distinguished from other forms and types of arch obstruction. Among the 489 patients without aortic arch obstruction, two-dimensional echocardiography wrongly diagnosed the presence of such obstruction in 9 patients (overall specificity 98%). Forty-six (92%) of the 51 patients had at least one associated intracardiac abnormality. Twenty-two (44%) had a ventricular septal defect, 21 (42%) a bicuspid aortic valve and 4 (18%) subaortic stenosis. Five patients had complex congenital cardiac malformations. All associated abnormalities were prospectively identified with two-dimensional echocardiography. Thus, two-dimensional echocardiography is highly specific in diagnosing aortic arch obstruction. It is less sensitive for the diagnosis of isthmic hypoplasia in the neonatal period.

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