Surgical Treatment of Isolated Interrupted Aortic Arch in a Young Female Diagnosed during Pregnancy Mehmet Tas¸ar, Nur Dikmen Yaman, Zeynep Eyileten, and Adnan Uysalel, Ankara, Turkey
Interrupted aortic arch (IAA) is a very rare pathology characterized by luminal discontinuity between ascending and descending aorta. IAA is commonly treated in pediatric ages, but the surgery is rarely used for adult patients. In this case report, we aimed to present a successful surgery in a young woman in whom IAA was diagnosed during pregnancy.
Interrupted aortic arch (IAA) is a congenital malformation with 3 distinct types, characterized by complete luminal dissociation between the ascending and descending aorta.1 It is very rarely reported in adults because this congenital malformation is typically diagnosed and repaired during the neonatal period. We discuss the presentation and surgical treatment of a young woman with a diagnosis of isolated IAA after her first pregnancy.
CASE REPORT A 25-year-old woman, with history of hypertension and syncopal episodes during her first pregnancy, was referred to cardiology department at 34 weeks of gestation for further investigations for suspicion of secondary arterial hypertension in the course of congenital heart disease. On her admission, her blood pressures were 140/90 mm Hg in the upper limbs and 90/60 mm Hg in the lower limbs. The pulses were equal over both upper extremities, and radiofemoral
There is no conflict of interest or financial support related to this article. Cardiovascular Surgery Department, School of Medicine, Ankara University, Ankara, Turkey. Correspondence to: Mehmet Tas¸ar, MD, Cardiovascular Surgery Department, School of Medicine, Ankara University, Dikimevi, Ankara 06340, Turkey; E-mail: [email protected] Ann Vasc Surg 2015; 29: 842.e15e842.e17 http://dx.doi.org/10.1016/j.avsg.2014.12.032 Ó 2015 Elsevier Inc. All rights reserved. Manuscript received: October 13, 2014; manuscript accepted: December 9, 2014; published online: February 27, 2015.
delay was noted. The bilateral femoral and popliteal pulses were extremely weak, and the dorsalis pedis and anterior tibial pulses were impalpable. Transthoracic echocardiography showed aortic coarctation before the origin of subclavian artery causing the gradient of 42 mm Hg and left ventricular hypertrophy (Fig. 1). Because of pregnancy, magnetic resonance imaging (MRI) was preferred and determined aortic arch interruption measured 2 mm, after separation of left common carotid artery. Under antihypertensive treatment and close follow-up, she delivered a healthy baby via normal spontaneous vaginal way without complications. After puerperal period, she referred to our clinic for surgical repair. First, computerized tomography scan was used to show the details of pathology, and findings were compatible with MRI. She underwent end-to-side bypass grafting with 15-cm-long polytetraflourethylene (PTFE No:18) graft that was anastomosed between descending and ascending aorta under cardiopulmonary bypass. The patient’s postoperative recovery was uneventful. On examination at 30 days after operation, she had no symptoms and control computerized tomographic angiography at that time showed a patent graft (Fig. 2). After the operation, blood pressure was in normal values so there was no need to antihypertensive drugs such as on 1-year follow-up.
DISCUSSION IAA is characterized by a discontinuity between the ascending and descending intrathoracic aorta. The 842.e15
842.e16 Case reports
Annals of Vascular Surgery
Fig. 2. Arrow shows the patent graft between ascending and descending aorta.
Fig. 1. Arrow shows the interrupted segment of the aorta.
lack of continuity may be either complete or incomplete in form of a nonpatent fibrotic band in the transverse arch or aortic isthmus. IAA is a rare duct-dependent anomaly, representing about 1.5% of cardiac malformations occurring in approximately 2 of 100,000 live births.2 IAA is associated with additional cardiovascular anatomic defects in up to 98% of cases. Rarely, IAA is an isolated finding without another associated cardiac defect. This lesion is often associated with ventricular septal defect, subaortic stenosis, or persistent truncus arteriosus. In 1959, Celoria and Patton3 developed the following classification: type A (IAA-A): interruption between the left subclavian artery and descending aorta; type B (IAA-B): interruption between the left subclavian artery and left common carotid artery; and type C (IAA-C): interruption between the innominate and left carotid artery. The most common form is IAA-B (84%), followed by IAA-A (13%) and IAA-C (3%).4 The hemodynamics depends on patency of ductus and associated heart lesions.5 The mortality and long-term results after repair of IAA depend on morphology, clinical status, surgical methods, and experience.6 IAA is very rarely reported in adults because this congenital malformation is typically diagnosed and repaired during the neonatal period. An untreated
individual’s survival to adulthood depends on the development of adequate collateral blood flow. Most adults with IAA reportedly have a type A interruption and no ventricular septal defect.7 Seventy percent of patients had hypertension refractory to medical management as their presenting symptom. Other common presenting symptoms were claudication (13%), congestive heart failure (6%), and aortic insufficiency (10%). Lifethreatening complications were present at initial presentation in 13% of patients. These included coronary artery disease, intracranial hemorrhage, and biventricular heart failure. Surgical correction for IAA can be accomplished through either a staged approach or a single surgery. For both strategies, risks of mortality and reintervention have been described. Although it is extremely rare, IAA must be considered along with aortic coarctation in the differential diagnosis of an adult patient with hypertension refractory to medical management and diminished pulses in the lower extremities. Surgical intervention is necessary both to improve the patient’s symptomatology and to prevent potentially fatal sequelae. REFERENCES 1. Gordon E, Person T, Kavarana M, et al. Interrupted aortic arch in the adult. J Card Surg 2011;26:405e9. 2. Fliedner R, Kawecki A, Enzensberger C, et al. Fetal and neonatal diagnosis of interrupted aortic arch: associations and outcomes. Fetal Diagn Ther 2011;30:299e305. 3. Celoria GC, Patton RB. Congenital absence of the aortic arch. Am Heart J 1959;58:408. 4. Schreiber C, Mazzitelli D, Haehnel JC, et al. The interrupted aortic arch: an overview after 20 years of surgical treatment. Eur J Cardiothorac Surg 1997;12:466e9.
Vol. 29, No. 4, May 2015
5. Tlaskal T, Vojtovic P, Reich O, et al. Improved results after the primary repair of interrupted aortic arch: impact of a new management protocol with isolated cerebral perfusion. Eur J Cardiothorac Surg 2010;38:52e8.
Case reports 842.e17
6. Mishra PK. Management strategies for interrupted aortic arch with associated anomalies. Eur J Cardiothorac Surg 2009;35:569e76. 7. Borgohain S, Gupta A, Grover V, Gupta V. Isolated interrupted aortic arch in an 18-year-old man. Tex Heart Inst J 2013;40:79e81.
Interrupted aortic arch (IAA) is a very rare pathology characterized by luminal discontinuity between ascending and descending aorta. IAA is commonly treated in pediatric ages, but the surgery is rarely used for adult patients. In this case report, we aimed to present a successful surgery in a young woman in whom IAA was diagnosed during pregnancy.
Interrupted aortic arch (IAA) is a congenital malformation with 3 distinct types, characterized by complete luminal dissociation between the ascending and descending aorta.1 It is very rarely reported in adults because this congenital malformation is typically diagnosed and repaired during the neonatal period. We discuss the presentation and surgical treatment of a young woman with a diagnosis of isolated IAA after her first pregnancy.
CASE REPORT A 25-year-old woman, with history of hypertension and syncopal episodes during her first pregnancy, was referred to cardiology department at 34 weeks of gestation for further investigations for suspicion of secondary arterial hypertension in the course of congenital heart disease. On her admission, her blood pressures were 140/90 mm Hg in the upper limbs and 90/60 mm Hg in the lower limbs. The pulses were equal over both upper extremities, and radiofemoral
There is no conflict of interest or financial support related to this article. Cardiovascular Surgery Department, School of Medicine, Ankara University, Ankara, Turkey. Correspondence to: Mehmet Tas¸ar, MD, Cardiovascular Surgery Department, School of Medicine, Ankara University, Dikimevi, Ankara 06340, Turkey; E-mail: [email protected] Ann Vasc Surg 2015; 29: 842.e15e842.e17 http://dx.doi.org/10.1016/j.avsg.2014.12.032 Ó 2015 Elsevier Inc. All rights reserved. Manuscript received: October 13, 2014; manuscript accepted: December 9, 2014; published online: February 27, 2015.
delay was noted. The bilateral femoral and popliteal pulses were extremely weak, and the dorsalis pedis and anterior tibial pulses were impalpable. Transthoracic echocardiography showed aortic coarctation before the origin of subclavian artery causing the gradient of 42 mm Hg and left ventricular hypertrophy (Fig. 1). Because of pregnancy, magnetic resonance imaging (MRI) was preferred and determined aortic arch interruption measured 2 mm, after separation of left common carotid artery. Under antihypertensive treatment and close follow-up, she delivered a healthy baby via normal spontaneous vaginal way without complications. After puerperal period, she referred to our clinic for surgical repair. First, computerized tomography scan was used to show the details of pathology, and findings were compatible with MRI. She underwent end-to-side bypass grafting with 15-cm-long polytetraflourethylene (PTFE No:18) graft that was anastomosed between descending and ascending aorta under cardiopulmonary bypass. The patient’s postoperative recovery was uneventful. On examination at 30 days after operation, she had no symptoms and control computerized tomographic angiography at that time showed a patent graft (Fig. 2). After the operation, blood pressure was in normal values so there was no need to antihypertensive drugs such as on 1-year follow-up.
DISCUSSION IAA is characterized by a discontinuity between the ascending and descending intrathoracic aorta. The 842.e15
842.e16 Case reports
Annals of Vascular Surgery
Fig. 2. Arrow shows the patent graft between ascending and descending aorta.
Fig. 1. Arrow shows the interrupted segment of the aorta.
lack of continuity may be either complete or incomplete in form of a nonpatent fibrotic band in the transverse arch or aortic isthmus. IAA is a rare duct-dependent anomaly, representing about 1.5% of cardiac malformations occurring in approximately 2 of 100,000 live births.2 IAA is associated with additional cardiovascular anatomic defects in up to 98% of cases. Rarely, IAA is an isolated finding without another associated cardiac defect. This lesion is often associated with ventricular septal defect, subaortic stenosis, or persistent truncus arteriosus. In 1959, Celoria and Patton3 developed the following classification: type A (IAA-A): interruption between the left subclavian artery and descending aorta; type B (IAA-B): interruption between the left subclavian artery and left common carotid artery; and type C (IAA-C): interruption between the innominate and left carotid artery. The most common form is IAA-B (84%), followed by IAA-A (13%) and IAA-C (3%).4 The hemodynamics depends on patency of ductus and associated heart lesions.5 The mortality and long-term results after repair of IAA depend on morphology, clinical status, surgical methods, and experience.6 IAA is very rarely reported in adults because this congenital malformation is typically diagnosed and repaired during the neonatal period. An untreated
individual’s survival to adulthood depends on the development of adequate collateral blood flow. Most adults with IAA reportedly have a type A interruption and no ventricular septal defect.7 Seventy percent of patients had hypertension refractory to medical management as their presenting symptom. Other common presenting symptoms were claudication (13%), congestive heart failure (6%), and aortic insufficiency (10%). Lifethreatening complications were present at initial presentation in 13% of patients. These included coronary artery disease, intracranial hemorrhage, and biventricular heart failure. Surgical correction for IAA can be accomplished through either a staged approach or a single surgery. For both strategies, risks of mortality and reintervention have been described. Although it is extremely rare, IAA must be considered along with aortic coarctation in the differential diagnosis of an adult patient with hypertension refractory to medical management and diminished pulses in the lower extremities. Surgical intervention is necessary both to improve the patient’s symptomatology and to prevent potentially fatal sequelae. REFERENCES 1. Gordon E, Person T, Kavarana M, et al. Interrupted aortic arch in the adult. J Card Surg 2011;26:405e9. 2. Fliedner R, Kawecki A, Enzensberger C, et al. Fetal and neonatal diagnosis of interrupted aortic arch: associations and outcomes. Fetal Diagn Ther 2011;30:299e305. 3. Celoria GC, Patton RB. Congenital absence of the aortic arch. Am Heart J 1959;58:408. 4. Schreiber C, Mazzitelli D, Haehnel JC, et al. The interrupted aortic arch: an overview after 20 years of surgical treatment. Eur J Cardiothorac Surg 1997;12:466e9.
Vol. 29, No. 4, May 2015
5. Tlaskal T, Vojtovic P, Reich O, et al. Improved results after the primary repair of interrupted aortic arch: impact of a new management protocol with isolated cerebral perfusion. Eur J Cardiothorac Surg 2010;38:52e8.
Case reports 842.e17
6. Mishra PK. Management strategies for interrupted aortic arch with associated anomalies. Eur J Cardiothorac Surg 2009;35:569e76. 7. Borgohain S, Gupta A, Grover V, Gupta V. Isolated interrupted aortic arch in an 18-year-old man. Tex Heart Inst J 2013;40:79e81.
