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Double aortic arch associated with coarctation
258
IJC 0434B
Double aortic arch associated with coarctation Josh A. Ettedgui *, Avraham Lorber 1 and David Anderson * Departments of
’ Paediatnc Cardiologv and ’ Cardiothoracic Surgery, Guy’s Hospital, London, U.K. (Received and accepted 6 February 1986)
An unusual combination of double aortic arch with coarctation of one of its limbs is described. We emphasize the importance of preoperative aortography to demonstrate or exclude coarctation which is clinically silent prior to surgery. This rare association will determine the surgical approach. (Key words:
double aortic arch; coarctation:
aortography)
Double aortic arch is a well recognized cause of oesophageal and of tracheal compression. Most frequently it is an isolated anomaly but a large minority of affected patients have other cardiovascular malformations. The most common of these is the tetralogy of Fallot [l]. In this communication we report a case in which double aortic arch was associated with coarctation of one of the limbs. This combination, although rare [2,3], has important diagnostic and therapeutic implications. Case Report J.G. was noted to have noisy breathing from birth. Despite having minor feeding difficulties her weight gain was adequate. By the age of three months it became evident that she had stridor. A barium swallow at that time showed a posterior indentation of the oesophagus which suggested the diagnosis of a vascular ring with an aberrant right subclavian artery. Physical examination at five months of age showed a happy, active child whose only detectable abnormality was mild stridor. Her weight was 7.75 kg. She had normal upper and lower limb pulses with an upper limb blood pressure of 80/50 mm Hg. The apex beat was in the fifth intercostal space in the midclavicular line and the cardiac impulse was normal. On auscultation the first and second heart sounds were normal and there were no murmurs. Cross-sectional echocardiography showed normal intracardiac anatomy and a double aortic arch with an underdeveloped left sided component. A retrograde aortogram confirmed the echocardiographic diagnosis and revealed a coarctation of the left arch distal to the origin of the left subclavian artery (Fig. 1). She subsequently underwent successful surgical correction during which the left arch was divided proximal to the left subclavian artery and both ends oversewn. The left-sided arterial ligament (ligamentum arteriosum) which connected the left subclavian artery to the left pulmonary artery, was also divided and oversewn. This completely relieved the compressive effect of the vascular ring. The left subclavian artery remained arising from the descending portion of the right aortic arch with the coarcted
Correspondence to: Department of Paediatric Cardiology, 11th Floor Guy’s Tower. Guy’s Hospital, St. Thomas Street, London SE1 9RT. U.K. International Journal of Cardiolog): 12 (1986) 258-260 0 Elsevier Science Publishers B.V. (Biomedical Division)
259
Fig. 1. Lateral view of a retrograde hypoplastic left limb and the associated
aortogram coarctation.
that
demonstrates
the double
aortic
portion at its origin. Surgical attempts to relieve this stenosis were not made. diminished left arm pulses, this has not had any detectable clinical effect.
arch
Apart
with
a
from
Discussion Coarctation occurring in one limb of a double aortic arch is a silent lesion. There are no signs or symptoms attributable to the obstruction. Adverse haemodynamic effects become apparent only when the unobstructed limb has been divided. There was no clinical suspicion of coarctation in the case reported here. All the pulses were normal prior to surgery. It was not until the left arch was divided that the left bra&al pulses became weak. Aortography alone indicated the presence of coexisting coarctation in this patient. Failure to perform a preoperative aortogram may have dire consequences. This probably contributed to the fatal outcome in one of the cases reported by Raju et al. [2] in which the unobstructed aortic arch was surgically divided. Postoperatively the child was anuric and the physical signs of
260
coarctation became obvious. An emergency aortogram confirmed the diagnosis but the patient did not survive the second operation. We emphasize the need for preoperative aortography in double aortic arch since it is only by this means that coarctation can be demonstrated or excluded. This is important since, when it is present, the associated coarctation will determine the surgical approach.
References 7 Binet JP. Langlois J. Aortic arch anomalies in children and infants. J Thorac Cardiovasc Surg 1977; 73: 248-252. 2 Raju S. Ratcliff J, Timmis H, Watson D, Suzuki A. Internal coarctation associated with double aortic arch. J Thorac Cardiovasc Surg 1973; 66: 192-195. 3 Merin G, Borman J. Aviad I, Maddock C, Stem S. Double aortic arch associated with coarctation. ventricular septal defect and right ventricular outflow tract obstruction. Successful surgical repair. Am J Cardiol 1972; 29: 564-567.
IJC 0434C
Limited effect of magnesium sulphate on torsades de pointes ventricular tachycardia L.K. Toivonen and H. Leinonen First Department
of Medicine, (Received
University Central Hospital, Helsinki,
and accepted
24 February
Finland
1986)
A patient with thioridazine-induced torsades de pointes ventricular tachycardia treated with magnesium sulphate is presented, Due to incessant recurrence of tachycardias it was possible to observe the time course of the effect of 1 g intravenous doses. Suppression of torsades de pointes lasted at best for no more than 18 min and a maintenance infusion was not effective without overdrive pacing. Magnesium sulphate may have value as first aid therapy for drug-induced torsades de pointes ventricular tachycardia. However, its effect disappears rapidly, and, therefore, should not alone be relied on as prophylactic treatment.
(Key words: ventricular arrhythmias;
magnesium)
Cases were recently reported suggesting that magnesium sulphate might be useful in the treatment of torsades de pointes ventricular tachycardia with different aetiologies [l-3]. We present a case with thioridazine-induced ventricular tachycardia treated with magnesium infusions. The beneficial effect was short-lasting.
Reprint requests to: Dr. Lauri Toivonen, M.D., Cardiovascular Hospital, Haartmaninkatu 4, SF-00290 Helsinki 29, Finland.
International Journal of Cardiology, 12 (1986) 260-262 Q Elsevier Science Publishers B.V. (Biomedical Division)
Laboratory,
University
Central
IJC 0434B
Double aortic arch associated with coarctation Josh A. Ettedgui *, Avraham Lorber 1 and David Anderson * Departments of
’ Paediatnc Cardiologv and ’ Cardiothoracic Surgery, Guy’s Hospital, London, U.K. (Received and accepted 6 February 1986)
An unusual combination of double aortic arch with coarctation of one of its limbs is described. We emphasize the importance of preoperative aortography to demonstrate or exclude coarctation which is clinically silent prior to surgery. This rare association will determine the surgical approach. (Key words:
double aortic arch; coarctation:
aortography)
Double aortic arch is a well recognized cause of oesophageal and of tracheal compression. Most frequently it is an isolated anomaly but a large minority of affected patients have other cardiovascular malformations. The most common of these is the tetralogy of Fallot [l]. In this communication we report a case in which double aortic arch was associated with coarctation of one of the limbs. This combination, although rare [2,3], has important diagnostic and therapeutic implications. Case Report J.G. was noted to have noisy breathing from birth. Despite having minor feeding difficulties her weight gain was adequate. By the age of three months it became evident that she had stridor. A barium swallow at that time showed a posterior indentation of the oesophagus which suggested the diagnosis of a vascular ring with an aberrant right subclavian artery. Physical examination at five months of age showed a happy, active child whose only detectable abnormality was mild stridor. Her weight was 7.75 kg. She had normal upper and lower limb pulses with an upper limb blood pressure of 80/50 mm Hg. The apex beat was in the fifth intercostal space in the midclavicular line and the cardiac impulse was normal. On auscultation the first and second heart sounds were normal and there were no murmurs. Cross-sectional echocardiography showed normal intracardiac anatomy and a double aortic arch with an underdeveloped left sided component. A retrograde aortogram confirmed the echocardiographic diagnosis and revealed a coarctation of the left arch distal to the origin of the left subclavian artery (Fig. 1). She subsequently underwent successful surgical correction during which the left arch was divided proximal to the left subclavian artery and both ends oversewn. The left-sided arterial ligament (ligamentum arteriosum) which connected the left subclavian artery to the left pulmonary artery, was also divided and oversewn. This completely relieved the compressive effect of the vascular ring. The left subclavian artery remained arising from the descending portion of the right aortic arch with the coarcted
Correspondence to: Department of Paediatric Cardiology, 11th Floor Guy’s Tower. Guy’s Hospital, St. Thomas Street, London SE1 9RT. U.K. International Journal of Cardiolog): 12 (1986) 258-260 0 Elsevier Science Publishers B.V. (Biomedical Division)
259
Fig. 1. Lateral view of a retrograde hypoplastic left limb and the associated
aortogram coarctation.
that
demonstrates
the double
aortic
portion at its origin. Surgical attempts to relieve this stenosis were not made. diminished left arm pulses, this has not had any detectable clinical effect.
arch
Apart
with
a
from
Discussion Coarctation occurring in one limb of a double aortic arch is a silent lesion. There are no signs or symptoms attributable to the obstruction. Adverse haemodynamic effects become apparent only when the unobstructed limb has been divided. There was no clinical suspicion of coarctation in the case reported here. All the pulses were normal prior to surgery. It was not until the left arch was divided that the left bra&al pulses became weak. Aortography alone indicated the presence of coexisting coarctation in this patient. Failure to perform a preoperative aortogram may have dire consequences. This probably contributed to the fatal outcome in one of the cases reported by Raju et al. [2] in which the unobstructed aortic arch was surgically divided. Postoperatively the child was anuric and the physical signs of
260
coarctation became obvious. An emergency aortogram confirmed the diagnosis but the patient did not survive the second operation. We emphasize the need for preoperative aortography in double aortic arch since it is only by this means that coarctation can be demonstrated or excluded. This is important since, when it is present, the associated coarctation will determine the surgical approach.
References 7 Binet JP. Langlois J. Aortic arch anomalies in children and infants. J Thorac Cardiovasc Surg 1977; 73: 248-252. 2 Raju S. Ratcliff J, Timmis H, Watson D, Suzuki A. Internal coarctation associated with double aortic arch. J Thorac Cardiovasc Surg 1973; 66: 192-195. 3 Merin G, Borman J. Aviad I, Maddock C, Stem S. Double aortic arch associated with coarctation. ventricular septal defect and right ventricular outflow tract obstruction. Successful surgical repair. Am J Cardiol 1972; 29: 564-567.
IJC 0434C
Limited effect of magnesium sulphate on torsades de pointes ventricular tachycardia L.K. Toivonen and H. Leinonen First Department
of Medicine, (Received
University Central Hospital, Helsinki,
and accepted
24 February
Finland
1986)
A patient with thioridazine-induced torsades de pointes ventricular tachycardia treated with magnesium sulphate is presented, Due to incessant recurrence of tachycardias it was possible to observe the time course of the effect of 1 g intravenous doses. Suppression of torsades de pointes lasted at best for no more than 18 min and a maintenance infusion was not effective without overdrive pacing. Magnesium sulphate may have value as first aid therapy for drug-induced torsades de pointes ventricular tachycardia. However, its effect disappears rapidly, and, therefore, should not alone be relied on as prophylactic treatment.
(Key words: ventricular arrhythmias;
magnesium)
Cases were recently reported suggesting that magnesium sulphate might be useful in the treatment of torsades de pointes ventricular tachycardia with different aetiologies [l-3]. We present a case with thioridazine-induced ventricular tachycardia treated with magnesium infusions. The beneficial effect was short-lasting.
Reprint requests to: Dr. Lauri Toivonen, M.D., Cardiovascular Hospital, Haartmaninkatu 4, SF-00290 Helsinki 29, Finland.
International Journal of Cardiology, 12 (1986) 260-262 Q Elsevier Science Publishers B.V. (Biomedical Division)
Laboratory,
University
Central