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Absent Pulmonary Valve Syndrome with Tetralogy of Fallot: A Rare Radiographic Presentation The syndrome of absent pulmonary valve is a rare congenital condition. When it does occur, it is often in conjunction with tetralogy of Fallot. We report herein a newborn with this anomaly, presenting with an unusual radiographic finding, unilateral opacification of the entire right lung. Case Report A 2940 g infant of a full-term uncomplicated pregnancy, developed cyanosis soon after birth, which progressed into overt respiratory failure requiring mechanical ventilatory support. Presence of congenital heart disease was suspected and the baby was referred to this institution for further analysis and treatment. Physical examination showed a cyanotic baby in respiratory distress. No obvious surface anomaly was noted. Right ventricular impulse was palpated, and a loud grade IV to-and-fro murmur was audible at the left sternal border. The liver was palpable 3 cm below the right costal margin, and the spleen was not palpable. Electrocardiogram (ECG) showed right axis deviation and prominent right ventricular forces. The chest radiogram showed unilateral opacification of the entire right lung and a nonspecific moderate cardiomegaly. The left pulmonary arterial tree was not prominent. Real-time cross-sectional ecbocardiogram demonstrated situs solitus with right ventricular hyupertrophy, large ventricular septal defect, overriding of the aorta, secundum atrial septal defect, and an absence of the pulmonary valve with a restrictive pulmonary artery ring. Doppler echocardiography, with the sampling volume positioned just above the pulmonary annulus, demonstrated a typical to-and-fro pattern with wide frequency distribution compatible with pulmonary stenosis insufficiency. The diagnosis of absent pulmonary valve with tetralogy of Fallot was made and operative intervention planned. Despite vigorous medical treatment, the baby did not survive and an autopsy was performed 1 h postmortem. Autopsy showed a situs solitus heart with normal lobulation of the lungs. A single spleen weighing 4g was located in its normal position. The main pulmonary artery and its main branches showed typical aneurysmal dilatations. The right pulmonary artery, measuring 14 mm in maximal diameter, was twice as large as the left one. The left pulmonary veins merged to form a single unobstructed drainage into the left atrium. There were two right pulmonary veins entering the left atrium. The upper vein was formed by fusion of the veins draining the upper and the middle right lobes. The pulmonary vein draining the upper right lobe traversed on the ventral aspect of the branch of the right pulmonary artery, which supplied the middle and the lower lobes, which was 8 mm in diameter. The pulmonary vein draining the middle lobe emerged from the pulmonary parenchyma to lie close to this aneurysmally dilated branch pulmonary artery. The lower lobe right pulmonary vein ran an unobstructed course. The systemic venous connections were normal, lntracardiac examination confirmed tetralogy of Fallot, secundum atrial septal defect, and absent pulmonary valve.
To the best of our knowledge, this is the first reported case of this syndrome, presenting as de-
Letters to the Editor
scribed. Autopsy findings suggest that pulmonary venous obstruction, caused by an aneurysmally dilated branch pulmonary artery, was the responsible mechanism. Prompt recognition of the underlying cardiac defects is imperative in this congenital condition, with early onset of respiratory symptoms. Pediatricians should be aware of this peculiar variant of radiographic presentation. Rousei Hamada Kiyomi Fukushima Tsuyoshi Oikawa Hiroyuki Ida Zenji Nonaka Kihei Maekawa Department of Pediatrics The Tokyo Jikei University School of Medicine Tokyo, Japan
P u l m o n a r y V a s c u l a r Sling In the article by Gikonyo et al. (1989), "Pulmonary vascular sling: Report of seven cases and review of the literature" (Pediatr Cardiol 10:81-89), the authors, in their literature review, overlooked two articles [1, 2]. The case reported by Lochard et al. [2] is especially interesting. A girl, aged 7 months, presented with stridor since birth. Chest x-ray demonstrated hyperexpansion of the right lung and barium swallow revealed anterior compression of the esophagus. At operation, the right bronchus was divided and a left pulmonary arteriopexy performed, followed by an end-to-end resuturing of the bronchus. The patient is now 28 years old, has three children, and is doing well. Lochard was the first to describe this unusual but effective technique of bronchial transection for primary one-stage correction of a pulmonary vascular sling. References 1. Langlois J, Binet JP, Planche C, Conso JF, Razafinombana A (1978) Chirurgie 104:58-73 2. Locbard J, Vert P, Chalnot P (1963) Ann Chir Thorac Cardiovasc 2:458-461
J.-C. Hoeffel HOpital Jeanne-D'Arc University o f N a n c y Nancy, France
Absent Pulmonary Valve Syndrome with Tetralogy of Fallot: A Rare Radiographic Presentation The syndrome of absent pulmonary valve is a rare congenital condition. When it does occur, it is often in conjunction with tetralogy of Fallot. We report herein a newborn with this anomaly, presenting with an unusual radiographic finding, unilateral opacification of the entire right lung. Case Report A 2940 g infant of a full-term uncomplicated pregnancy, developed cyanosis soon after birth, which progressed into overt respiratory failure requiring mechanical ventilatory support. Presence of congenital heart disease was suspected and the baby was referred to this institution for further analysis and treatment. Physical examination showed a cyanotic baby in respiratory distress. No obvious surface anomaly was noted. Right ventricular impulse was palpated, and a loud grade IV to-and-fro murmur was audible at the left sternal border. The liver was palpable 3 cm below the right costal margin, and the spleen was not palpable. Electrocardiogram (ECG) showed right axis deviation and prominent right ventricular forces. The chest radiogram showed unilateral opacification of the entire right lung and a nonspecific moderate cardiomegaly. The left pulmonary arterial tree was not prominent. Real-time cross-sectional ecbocardiogram demonstrated situs solitus with right ventricular hyupertrophy, large ventricular septal defect, overriding of the aorta, secundum atrial septal defect, and an absence of the pulmonary valve with a restrictive pulmonary artery ring. Doppler echocardiography, with the sampling volume positioned just above the pulmonary annulus, demonstrated a typical to-and-fro pattern with wide frequency distribution compatible with pulmonary stenosis insufficiency. The diagnosis of absent pulmonary valve with tetralogy of Fallot was made and operative intervention planned. Despite vigorous medical treatment, the baby did not survive and an autopsy was performed 1 h postmortem. Autopsy showed a situs solitus heart with normal lobulation of the lungs. A single spleen weighing 4g was located in its normal position. The main pulmonary artery and its main branches showed typical aneurysmal dilatations. The right pulmonary artery, measuring 14 mm in maximal diameter, was twice as large as the left one. The left pulmonary veins merged to form a single unobstructed drainage into the left atrium. There were two right pulmonary veins entering the left atrium. The upper vein was formed by fusion of the veins draining the upper and the middle right lobes. The pulmonary vein draining the upper right lobe traversed on the ventral aspect of the branch of the right pulmonary artery, which supplied the middle and the lower lobes, which was 8 mm in diameter. The pulmonary vein draining the middle lobe emerged from the pulmonary parenchyma to lie close to this aneurysmally dilated branch pulmonary artery. The lower lobe right pulmonary vein ran an unobstructed course. The systemic venous connections were normal, lntracardiac examination confirmed tetralogy of Fallot, secundum atrial septal defect, and absent pulmonary valve.
To the best of our knowledge, this is the first reported case of this syndrome, presenting as de-
Letters to the Editor
scribed. Autopsy findings suggest that pulmonary venous obstruction, caused by an aneurysmally dilated branch pulmonary artery, was the responsible mechanism. Prompt recognition of the underlying cardiac defects is imperative in this congenital condition, with early onset of respiratory symptoms. Pediatricians should be aware of this peculiar variant of radiographic presentation. Rousei Hamada Kiyomi Fukushima Tsuyoshi Oikawa Hiroyuki Ida Zenji Nonaka Kihei Maekawa Department of Pediatrics The Tokyo Jikei University School of Medicine Tokyo, Japan
P u l m o n a r y V a s c u l a r Sling In the article by Gikonyo et al. (1989), "Pulmonary vascular sling: Report of seven cases and review of the literature" (Pediatr Cardiol 10:81-89), the authors, in their literature review, overlooked two articles [1, 2]. The case reported by Lochard et al. [2] is especially interesting. A girl, aged 7 months, presented with stridor since birth. Chest x-ray demonstrated hyperexpansion of the right lung and barium swallow revealed anterior compression of the esophagus. At operation, the right bronchus was divided and a left pulmonary arteriopexy performed, followed by an end-to-end resuturing of the bronchus. The patient is now 28 years old, has three children, and is doing well. Lochard was the first to describe this unusual but effective technique of bronchial transection for primary one-stage correction of a pulmonary vascular sling. References 1. Langlois J, Binet JP, Planche C, Conso JF, Razafinombana A (1978) Chirurgie 104:58-73 2. Locbard J, Vert P, Chalnot P (1963) Ann Chir Thorac Cardiovasc 2:458-461
J.-C. Hoeffel HOpital Jeanne-D'Arc University o f N a n c y Nancy, France
![[Tetralogy of Fallot with absent pulmonary valve].](/assets/img/hold.png)
