Scand J Thor Cardiovasc Surg 26: 93-96, 1992
DUCTUS ARTERIOSUS IN PULMONARY ATRESIA WITH AND WITHOUT VENTRICULAR SEPTAL DEFECT Anatomic and Functional Differences Bruno Marino, Paolo Guccione, Adriano Carotti, Andrea De Zorzi, Roberto Di Donato and Carlo Marcelletti From the Departments of Pediatric Cardiology and Cardiac Surgery, Bambino Gesu’ Hospital, Rome, Italy
Scand Cardiovasc J Downloaded from informahealthcare.com by Queen's University on 01/02/15 For personal use only.
(Accepted for publication August 19, 1991)
Abstract. The pulmonary circulation is dependent on the ductus arteriosus in all patients with pulmonary atresia and intact ventricular septum and in some with pulmonary atresia and ventricular septal defect (tetralogy of Fallot type). To assess the time of ductal closure in these two patient categories, we compared the ages at first operation in 58 patients with pulmonary atresia and intact ventricular septum and 32 with pulmonary atresia and septal defect. The age distribution differed significantly between the groups. Whereas 90% of the children with intact ventricular septum required surgery in the first week of life, 50% of those with ventricular septal defect underwent surgery after the first month and 25% after the third month. The previously described and now confirmed anatomic differences of ductus arteriosus or different levels of endogenous prostaglandins may explain persistent patency of the ductus in pulmonary atresia with ventricular septal defect. The phenomenon may have important clinical implications regarding the timing and choke of surgical procedure. Key words: Pulmonary atresia, ventricular septal defect, ductus arteriosus.
The natural history of the ductus arteriosus in the setting of congenital heart disease with ductus-dependent pulmonary circulation is not fully known. Spontaneous closure of the ductus occurs at ‘neonatal age’, but the exact time has not been defined. The different morphology of the ductus arteriosus in cases of pulmonary atresia without versus with ventricular septal defect (tetralogy of Fallot type) was earlier described (4,7). When the septum is intact, the ductus is in continuity with the descending aorta at an obtuse angle, as in normal subjects. When there is septal defect, the ductus is in continuity with the aortic arch, and its angle with the descending aorta is acute
(4,7). These observations suggest differing etiology for the two types of pulmonary atresia-early embryogenesis when the defect is combined with ventricular septal defect and late development when the septum is intact (4, 7). Our study aimed to confirm this anatomic difference between the two groups of patients and to appraise its influence on clinical behavior and time of surgical presentation. MATERIAL AND METHODS We reviewed the clinical records of 90 consecutive patients with pulmonary atresia and ductus-dependent pulmonary circulation who underwent surgery in our Department. In 58 cases (group I) the interventricular septum was intact and in 32 (group 11) there was a ventricular septal defect (VSD). Only patients with confluent pulmonary ateries, single ductus arteriosus and wholly ductus-dependent circulation were included in the study. Those with systemic collateral arteries, other types of pulmonary blood supply (8) and previous operations were excluded. Since 36 patients underwent surgery on the basis of echocardiography alone (5, 6), angiocardiographies were available in 54 cases, 2 6 with intact ventricular septum and 28 with VSD (45% and 88% of the respective groups). The angle of the ductus arteriosus with the aorta was evaluated in lateral or left oblique projection as previously reported (7). The indication for operation in all cases was severe cyanosis with arterial p 0 2 < 28 mmHg. Because of metabolic acidosis, 4 6 (79%) of the group I (intact septum) and 8 (25 Yo) of the group I1 (VSD) patients needed prostaglandin treatment preoperatively. In 51 of the 58 patients with intact ventricular septum, systemic to pulmonary artery shunt was performed, associated in nine cases with pulmonary valvulotomy. The other seven had valvulotomy alone. Of the 32 patients with VSD, 28 underwent systemic Scand J Thoracic 26
Scand Cardiovasc J Downloaded from informahealthcare.com by Queen's University on 01/02/15 For personal use only.
94
B. Marino et al.
Fig. 1. Aortography in lateral projection in a patient with pulmonary atresia and intact ventricular septum. Note the obtuse angle of the ductus arteriosus (D) with the descending aorta (A).
Fig. 2. Aortography in left oblique projection in a patient with pulmonary atresia and ventricular septal defect. Note the acute angle of the ductus arteriosus (D) with the descending aorta (A). * = left pulmonary artery.
to pulmonary artery shunt, and in four a right ventricular outflow patch was inserted in an attempt to enlarge their diminutive pulmonary arteries. The ages of the two groups at surgery were compared. Statistical analysis. Means and standard deviations were computed for each group and analyzed with Student’s test. Z test was used to analyze differences of prevalence. Statistical significance was accepted as ~~0.05.
and showed an acute angle with the descending aorta (Fig. 2).
RESULTS
Angle of ductus arteriosus In all patients in group I (with infact ventricular septum) the ductus arteriosus formed an obtuse angle with the descending aorta (Fig. 1). In all the group I1 patients (with VSD), however, the ductus was in continuity with the aortic arch
Presentation of cyanosis and age at surgery Of the 58 children with pulmonary atresia and intact ventricular septum (group I), 52 (90%) presented severe cyanosis and needed surgery in the first week of life (Table I). The other six required operation between the seventh day and the third month. Severe symptoms appeared after the first month of life in only one child (1.7%). Ten (3 1 %) of the 32 patients with pulmonary atresia and VSD (group 11) were operated on in the first week of life, and six between days 7 and 30 (Table 1). In 16 children (50%) the arterial
Table I. Age at surgery in pulmonary atresia patients with ductus-dependent pulmonary circulation and intact ventricular septum or ventricular septal defect
Age at operation < 7 days 7-1 4 days 14-30 days 1- 3 months > 3 months Total Mean age (days) Median age (days) Scand J Thoracic 26
Group I Intact ventricular septum
Group I1 Ventricular septal defect
n
n
(Yo)
52
4 1 1 0 58
4.5 (SD 12.2) 4
(89.7) (6.9) (1.7) (1.7)
(Yo)
10 (3 1.2) 3 (9.4) (9.4) 3 8 (25.0) 8 (25.0) 32 206 (SD 72.5) 30
P < 0.000 1
NS NS
DUCTUS ARTERIOSUS IN PULMONARY ATRESIA WITH AND WITHOUT VENTRICULAR SEPTAL DEFECT Anatomic and Functional Differences Bruno Marino, Paolo Guccione, Adriano Carotti, Andrea De Zorzi, Roberto Di Donato and Carlo Marcelletti From the Departments of Pediatric Cardiology and Cardiac Surgery, Bambino Gesu’ Hospital, Rome, Italy
Scand Cardiovasc J Downloaded from informahealthcare.com by Queen's University on 01/02/15 For personal use only.
(Accepted for publication August 19, 1991)
Abstract. The pulmonary circulation is dependent on the ductus arteriosus in all patients with pulmonary atresia and intact ventricular septum and in some with pulmonary atresia and ventricular septal defect (tetralogy of Fallot type). To assess the time of ductal closure in these two patient categories, we compared the ages at first operation in 58 patients with pulmonary atresia and intact ventricular septum and 32 with pulmonary atresia and septal defect. The age distribution differed significantly between the groups. Whereas 90% of the children with intact ventricular septum required surgery in the first week of life, 50% of those with ventricular septal defect underwent surgery after the first month and 25% after the third month. The previously described and now confirmed anatomic differences of ductus arteriosus or different levels of endogenous prostaglandins may explain persistent patency of the ductus in pulmonary atresia with ventricular septal defect. The phenomenon may have important clinical implications regarding the timing and choke of surgical procedure. Key words: Pulmonary atresia, ventricular septal defect, ductus arteriosus.
The natural history of the ductus arteriosus in the setting of congenital heart disease with ductus-dependent pulmonary circulation is not fully known. Spontaneous closure of the ductus occurs at ‘neonatal age’, but the exact time has not been defined. The different morphology of the ductus arteriosus in cases of pulmonary atresia without versus with ventricular septal defect (tetralogy of Fallot type) was earlier described (4,7). When the septum is intact, the ductus is in continuity with the descending aorta at an obtuse angle, as in normal subjects. When there is septal defect, the ductus is in continuity with the aortic arch, and its angle with the descending aorta is acute
(4,7). These observations suggest differing etiology for the two types of pulmonary atresia-early embryogenesis when the defect is combined with ventricular septal defect and late development when the septum is intact (4, 7). Our study aimed to confirm this anatomic difference between the two groups of patients and to appraise its influence on clinical behavior and time of surgical presentation. MATERIAL AND METHODS We reviewed the clinical records of 90 consecutive patients with pulmonary atresia and ductus-dependent pulmonary circulation who underwent surgery in our Department. In 58 cases (group I) the interventricular septum was intact and in 32 (group 11) there was a ventricular septal defect (VSD). Only patients with confluent pulmonary ateries, single ductus arteriosus and wholly ductus-dependent circulation were included in the study. Those with systemic collateral arteries, other types of pulmonary blood supply (8) and previous operations were excluded. Since 36 patients underwent surgery on the basis of echocardiography alone (5, 6), angiocardiographies were available in 54 cases, 2 6 with intact ventricular septum and 28 with VSD (45% and 88% of the respective groups). The angle of the ductus arteriosus with the aorta was evaluated in lateral or left oblique projection as previously reported (7). The indication for operation in all cases was severe cyanosis with arterial p 0 2 < 28 mmHg. Because of metabolic acidosis, 4 6 (79%) of the group I (intact septum) and 8 (25 Yo) of the group I1 (VSD) patients needed prostaglandin treatment preoperatively. In 51 of the 58 patients with intact ventricular septum, systemic to pulmonary artery shunt was performed, associated in nine cases with pulmonary valvulotomy. The other seven had valvulotomy alone. Of the 32 patients with VSD, 28 underwent systemic Scand J Thoracic 26
Scand Cardiovasc J Downloaded from informahealthcare.com by Queen's University on 01/02/15 For personal use only.
94
B. Marino et al.
Fig. 1. Aortography in lateral projection in a patient with pulmonary atresia and intact ventricular septum. Note the obtuse angle of the ductus arteriosus (D) with the descending aorta (A).
Fig. 2. Aortography in left oblique projection in a patient with pulmonary atresia and ventricular septal defect. Note the acute angle of the ductus arteriosus (D) with the descending aorta (A). * = left pulmonary artery.
to pulmonary artery shunt, and in four a right ventricular outflow patch was inserted in an attempt to enlarge their diminutive pulmonary arteries. The ages of the two groups at surgery were compared. Statistical analysis. Means and standard deviations were computed for each group and analyzed with Student’s test. Z test was used to analyze differences of prevalence. Statistical significance was accepted as ~~0.05.
and showed an acute angle with the descending aorta (Fig. 2).
RESULTS
Angle of ductus arteriosus In all patients in group I (with infact ventricular septum) the ductus arteriosus formed an obtuse angle with the descending aorta (Fig. 1). In all the group I1 patients (with VSD), however, the ductus was in continuity with the aortic arch
Presentation of cyanosis and age at surgery Of the 58 children with pulmonary atresia and intact ventricular septum (group I), 52 (90%) presented severe cyanosis and needed surgery in the first week of life (Table I). The other six required operation between the seventh day and the third month. Severe symptoms appeared after the first month of life in only one child (1.7%). Ten (3 1 %) of the 32 patients with pulmonary atresia and VSD (group 11) were operated on in the first week of life, and six between days 7 and 30 (Table 1). In 16 children (50%) the arterial
Table I. Age at surgery in pulmonary atresia patients with ductus-dependent pulmonary circulation and intact ventricular septum or ventricular septal defect
Age at operation < 7 days 7-1 4 days 14-30 days 1- 3 months > 3 months Total Mean age (days) Median age (days) Scand J Thoracic 26
Group I Intact ventricular septum
Group I1 Ventricular septal defect
n
n
(Yo)
52
4 1 1 0 58
4.5 (SD 12.2) 4
(89.7) (6.9) (1.7) (1.7)
(Yo)
10 (3 1.2) 3 (9.4) (9.4) 3 8 (25.0) 8 (25.0) 32 206 (SD 72.5) 30
P < 0.000 1
NS NS
