- Email: [email protected]
Criss-cross heart with intact ventricular septum
382 patients on combined sotalol and flecainide treatment during the period of follow-up. One of them has satisfactorily improved after implantation of an antitachycardic pacemaker. Adverse effects during treatment were not observed.
occur. Of note is the lack of proarrhythmic effects during invasive testing which may strengthen the clinical value of this combination therapy. References Kunze KP. Schhiter M. Kuck KH. Sotalol in patients with Wolff-Parkinson-White syndrome. Circulation 1987:75: 1050~1057. Roden DM. Woo&s RL. Drug therapy. Flecainide. N Engl J Med 1986;315:36-41. Gtilker H. Hewer H, Frenking B. et al. Enhanced antiarrhythmic efficacy of combined sotalol/flecainide and combined sotalol/propafenone on chronic ventricular arrhythmia (abstract). Circulation 1984:70:11-444. Waldo AL, Akthar M. Benditt DG, et al. Appropriate electrophysiologic study and treatment of patients with the Wolff-Parkinson-White syndrome. Pace 1988;11:536-543. Feld GK. Nademanee K, Stevenson W, Weiss J. Khtzner T, Singh BN. Clinical and electrophysiologic effects of amiodarone in patients with atria1 fibrillation complicating the Wolff-Parkinson-White syndrome. Am Heart J 1988: 115:1022107.
Discussion The present study shows that this combination therapy given in low doses may be used safely in selected patients even after multiple failures of antiarrhythmic drugs. The electrophysiologic data reflect the role of both drugs [1,2]. The ability to impede satisfactorily fast conducting accessory pathways is an advantage which compares to treatment with single drugs such as sotalol or amiodarone given at high dosage [1,5]. The low plasma levels of both drugs do not suggest pharmacologic interaction to provide the basis for the electrophysiologic effects obtained by this combination therapy. Since the incidence of side effects relates to plasma trough levels for both drugs, adverse effects did not
Internatwnal
CARD10
Journal of Cardiologv, 26 (1990) 382-385 Elsevier
10347
Criss-cross Valmir F. Fontes,
heart with intact ventricular
septum
JosC Albino Malta de Souza and Sergio C. Pontes Jr.
Diwsion of Pediatric Cardiology and Hemo&namlc (Received
Laborato
29 August
1989: revision
accepted
Institute
1 November
of Cardioiogv. Sao Paula-SP,
Brazil
1989)
We report a case of a criss-cross heart in a 6-month-old male child with echocardiographic and angiocardiographic findings of concordant atrioventricular and discordant ventriculoarterial connexions and an intact ventricular septum. This entity has been described with a ventricular septal defect as part of its own anatomic spectrum. The concept of the criss-cross heart, however, is best restricted to a form of spatial atrioventricular arrangement in which the atrioventricular connexions are criss-crossed. Key words:
Criss-cross
heart;
Crossed
atrioventricular
Correspondence to: V.F. Fontes, M.D., Institute Pazzanese de Cardiologia, P.O. Box 215, Sao Paulo-SP. Brazil.
0167-5273/90/$03.50
ID1990 Elsevier Science Publishers
connexion
Dante 04012-
and relationship;
Ventricular
septal defect
The unusual atrioventricular arrangement in which there is crossing of atrioventricular connexions was
B.V. (Biomedical
Division)
383
originally reported by Lev and Rowlatt in 1961 [l]. The heart, however, was introduced by term “criss-cross” Anderson et al. [2] in 1974. Since then, several authors [3-51 have contributed to a better understanding of this bizarre spatial atrioventricular relationship. Thus far, cases have been found associated with a deficiency of ventricular septation. To the best of our knowledge cases without ventricular septal defects have not been reported in the English literature. We now report such a case. Case Report We report here a case of “criss-cross heart” with usual atria1 arrangement, concordant venoatrial and atrioventricular connexions, intact ventricular septum and discordant ventriculoarterial connexion in a cyanotic 6-month-old male child. Cyanosis and mild
congestive heart failure had been noted on the third day of life. As the clinical picture deteriorated, the child was hospitalized at 6 months of life in our institution for diagnosis and treatment. His haemoglobin was 16.8/dl and his arterial oxygen tension was 90 U torr. The electrocardiogram showed a heart rate of 150 beats per minute, left axis deviation, right atria1 enlargement and right ventricular hypertrophy. His chest X-ray revealed overfilled lung fields and a large cardiac silhouette, interpreted as due to enlargement and hypertrophy of the right heart chambers. An echocardiogram showed moderate right atria1 and right ventricular enlargement. The morphologic findings included usual atria1 arrangement (solitus), normal venoatrial connexion, crossed but concordant atrioventricular connexions, an intact ventricular septum and discordant ventriculoarterial connexions (Fig. 1). Catheterization revealed that the right ventricular pressure was systemic and the left
Fig. 1. Cross-sectional echocardiography discloses the major anatomic findings in apical longitudinal views. A. The atrioventricular connexion is concordant, the right atrium (RA) connecting to a superiorly located right ventricle (RV), while the left atrium (LA) connects to a inferiorly located left ventricle (LV). B. A redundant and very prominent floor of the oval fossa protrudes into the left ventricular in diastole. The left ventricular outflow tract supports the pulmonary trunk (PT) (Fig. B), and the right ventricular infundibulum supports the aorta (Ao) (Fig. D).
384
Fig. 2. Angiography confirms the criss-cross atrioventricular relationships. Note the superior and inferior ventricular relationshi ) and the crossed atrioventricular connexion (A and B). The aorta (Ao) arises from the right ventricle (RV), while the pulmonary trunk (W arises from the left ventricle (LV). The ventricular septum is intact (C and D).
ventricular pressure was about 50 mm Hg. The catheter was passed from the right into the left atrium through a moderate sized atria1 septal defect within the oval fossa. The oxygen saturation was 70% in the superior caval vein and 80% in the aortic root. Selective right and left ventriculography confirmed the echocardiographic findings (Fig. 2). The patient underwent a Senning type of
surgical repair in 1985. The follow-up is satisfactory he remains asymptomatic since his surgery.
and
Discussion
The morphologic spectrum of hearts with cro Nssed atrioventricular relationships includes a wide variet Y of
385 segmental arrangements. Although the venoatrial connexions are usually normal. the atrioventricular connexion can be either concordant or discordant. The ventricular chambers are almost always arranged in supero-inferior fashion, the right ventricle being superior while the left ventricle is inferiorly located. The ventriculoarterial connexion is commonly double outlet from the right ventricle, less often discordant and uncommonly concordant. Until now, a ventricular septal defect has always been an associated malformation. Other associated anomalies include hypoplasia or stenosis of the right ventricle, straddling and/or overriding of the atrioventricular valves, and subaortic or subpulmonary stenosis. To date. all these anatomic features have been relatively variable except for the presence of a ventricular septal defect. The present report demonstrates that a ventricular septal defect does not have to be present. The concept of the “criss-cross heart”, therefore should be confined to the description of a particular pattern of crossing of the atrioventricular connexions.
References Lev M. Rowlatt UF. The pathologic anatomy of mixed levocardia. A review of thirteen cases of atrial or ventricular inversion with or without corrected transposition. Am J Cardiol 1961;9:216-263. Anderson RH, Shinebourne EA. Gerlis LM. Crisscross atrioventricular relationships producing paradoxical atrioventricular concordance or discordance. Their significance to nomenclature of congenital heart disease. Circulation 1974:50:176-180. Kinsley RH, McGoon DC. Danielson GK. Corrected transposition of the great arteries associated with ventricular rotation. Circulation 1974;49:574-578. Anderson KR. Lie JT. Sieg K. Hagler DG, Davis GD. A criss-cross heart. Detailed anatomic description and discussion of morphogenesis. Mayo Clin Proc 1977;52:569-575. Symons JC, Shinebourne EA. Joseph MC, Lincoln C. Ho Y, Anderson RH. Criss-cross heart with congenitally corrected transposition: report of a case with d-transposed aorta and ventricular pre-excitation. Eur J Cardioi 1977;5:493-505.
occur. Of note is the lack of proarrhythmic effects during invasive testing which may strengthen the clinical value of this combination therapy. References Kunze KP. Schhiter M. Kuck KH. Sotalol in patients with Wolff-Parkinson-White syndrome. Circulation 1987:75: 1050~1057. Roden DM. Woo&s RL. Drug therapy. Flecainide. N Engl J Med 1986;315:36-41. Gtilker H. Hewer H, Frenking B. et al. Enhanced antiarrhythmic efficacy of combined sotalol/flecainide and combined sotalol/propafenone on chronic ventricular arrhythmia (abstract). Circulation 1984:70:11-444. Waldo AL, Akthar M. Benditt DG, et al. Appropriate electrophysiologic study and treatment of patients with the Wolff-Parkinson-White syndrome. Pace 1988;11:536-543. Feld GK. Nademanee K, Stevenson W, Weiss J. Khtzner T, Singh BN. Clinical and electrophysiologic effects of amiodarone in patients with atria1 fibrillation complicating the Wolff-Parkinson-White syndrome. Am Heart J 1988: 115:1022107.
Discussion The present study shows that this combination therapy given in low doses may be used safely in selected patients even after multiple failures of antiarrhythmic drugs. The electrophysiologic data reflect the role of both drugs [1,2]. The ability to impede satisfactorily fast conducting accessory pathways is an advantage which compares to treatment with single drugs such as sotalol or amiodarone given at high dosage [1,5]. The low plasma levels of both drugs do not suggest pharmacologic interaction to provide the basis for the electrophysiologic effects obtained by this combination therapy. Since the incidence of side effects relates to plasma trough levels for both drugs, adverse effects did not
Internatwnal
CARD10
Journal of Cardiologv, 26 (1990) 382-385 Elsevier
10347
Criss-cross Valmir F. Fontes,
heart with intact ventricular
septum
JosC Albino Malta de Souza and Sergio C. Pontes Jr.
Diwsion of Pediatric Cardiology and Hemo&namlc (Received
Laborato
29 August
1989: revision
accepted
Institute
1 November
of Cardioiogv. Sao Paula-SP,
Brazil
1989)
We report a case of a criss-cross heart in a 6-month-old male child with echocardiographic and angiocardiographic findings of concordant atrioventricular and discordant ventriculoarterial connexions and an intact ventricular septum. This entity has been described with a ventricular septal defect as part of its own anatomic spectrum. The concept of the criss-cross heart, however, is best restricted to a form of spatial atrioventricular arrangement in which the atrioventricular connexions are criss-crossed. Key words:
Criss-cross
heart;
Crossed
atrioventricular
Correspondence to: V.F. Fontes, M.D., Institute Pazzanese de Cardiologia, P.O. Box 215, Sao Paulo-SP. Brazil.
0167-5273/90/$03.50
ID1990 Elsevier Science Publishers
connexion
Dante 04012-
and relationship;
Ventricular
septal defect
The unusual atrioventricular arrangement in which there is crossing of atrioventricular connexions was
B.V. (Biomedical
Division)
383
originally reported by Lev and Rowlatt in 1961 [l]. The heart, however, was introduced by term “criss-cross” Anderson et al. [2] in 1974. Since then, several authors [3-51 have contributed to a better understanding of this bizarre spatial atrioventricular relationship. Thus far, cases have been found associated with a deficiency of ventricular septation. To the best of our knowledge cases without ventricular septal defects have not been reported in the English literature. We now report such a case. Case Report We report here a case of “criss-cross heart” with usual atria1 arrangement, concordant venoatrial and atrioventricular connexions, intact ventricular septum and discordant ventriculoarterial connexion in a cyanotic 6-month-old male child. Cyanosis and mild
congestive heart failure had been noted on the third day of life. As the clinical picture deteriorated, the child was hospitalized at 6 months of life in our institution for diagnosis and treatment. His haemoglobin was 16.8/dl and his arterial oxygen tension was 90 U torr. The electrocardiogram showed a heart rate of 150 beats per minute, left axis deviation, right atria1 enlargement and right ventricular hypertrophy. His chest X-ray revealed overfilled lung fields and a large cardiac silhouette, interpreted as due to enlargement and hypertrophy of the right heart chambers. An echocardiogram showed moderate right atria1 and right ventricular enlargement. The morphologic findings included usual atria1 arrangement (solitus), normal venoatrial connexion, crossed but concordant atrioventricular connexions, an intact ventricular septum and discordant ventriculoarterial connexions (Fig. 1). Catheterization revealed that the right ventricular pressure was systemic and the left
Fig. 1. Cross-sectional echocardiography discloses the major anatomic findings in apical longitudinal views. A. The atrioventricular connexion is concordant, the right atrium (RA) connecting to a superiorly located right ventricle (RV), while the left atrium (LA) connects to a inferiorly located left ventricle (LV). B. A redundant and very prominent floor of the oval fossa protrudes into the left ventricular in diastole. The left ventricular outflow tract supports the pulmonary trunk (PT) (Fig. B), and the right ventricular infundibulum supports the aorta (Ao) (Fig. D).
384
Fig. 2. Angiography confirms the criss-cross atrioventricular relationships. Note the superior and inferior ventricular relationshi ) and the crossed atrioventricular connexion (A and B). The aorta (Ao) arises from the right ventricle (RV), while the pulmonary trunk (W arises from the left ventricle (LV). The ventricular septum is intact (C and D).
ventricular pressure was about 50 mm Hg. The catheter was passed from the right into the left atrium through a moderate sized atria1 septal defect within the oval fossa. The oxygen saturation was 70% in the superior caval vein and 80% in the aortic root. Selective right and left ventriculography confirmed the echocardiographic findings (Fig. 2). The patient underwent a Senning type of
surgical repair in 1985. The follow-up is satisfactory he remains asymptomatic since his surgery.
and
Discussion
The morphologic spectrum of hearts with cro Nssed atrioventricular relationships includes a wide variet Y of
385 segmental arrangements. Although the venoatrial connexions are usually normal. the atrioventricular connexion can be either concordant or discordant. The ventricular chambers are almost always arranged in supero-inferior fashion, the right ventricle being superior while the left ventricle is inferiorly located. The ventriculoarterial connexion is commonly double outlet from the right ventricle, less often discordant and uncommonly concordant. Until now, a ventricular septal defect has always been an associated malformation. Other associated anomalies include hypoplasia or stenosis of the right ventricle, straddling and/or overriding of the atrioventricular valves, and subaortic or subpulmonary stenosis. To date. all these anatomic features have been relatively variable except for the presence of a ventricular septal defect. The present report demonstrates that a ventricular septal defect does not have to be present. The concept of the “criss-cross heart”, therefore should be confined to the description of a particular pattern of crossing of the atrioventricular connexions.
References Lev M. Rowlatt UF. The pathologic anatomy of mixed levocardia. A review of thirteen cases of atrial or ventricular inversion with or without corrected transposition. Am J Cardiol 1961;9:216-263. Anderson RH, Shinebourne EA. Gerlis LM. Crisscross atrioventricular relationships producing paradoxical atrioventricular concordance or discordance. Their significance to nomenclature of congenital heart disease. Circulation 1974:50:176-180. Kinsley RH, McGoon DC. Danielson GK. Corrected transposition of the great arteries associated with ventricular rotation. Circulation 1974;49:574-578. Anderson KR. Lie JT. Sieg K. Hagler DG, Davis GD. A criss-cross heart. Detailed anatomic description and discussion of morphogenesis. Mayo Clin Proc 1977;52:569-575. Symons JC, Shinebourne EA. Joseph MC, Lincoln C. Ho Y, Anderson RH. Criss-cross heart with congenitally corrected transposition: report of a case with d-transposed aorta and ventricular pre-excitation. Eur J Cardioi 1977;5:493-505.