Eur J Pediatr (1992) 151:748-750
European Journal of
Pediatrics
9 Springer-Verlag 1992
Pulmonary hypertension complicating a ventriculo-atrial shunt K. H a a s n o o t 1 and A. J, van Vught 2 1Paediatric Intensive Care Unit, Free University Hospital, P.O. Box 7057, NL-1007 MB Amsterdam, The Netherlands 2Paediatric Intensive Care Unit, University Children's Hospital "Het Wilhelmina Kinderziehkenhuis", Utrecht, The Netherlands Received July 22, 1991 / Accepted after revision February 16, 1992
Abstract. T h e use of v e n t r i c u l o - a t r i a l shunts for C S F div e r s i o n in d e v e l o p i n g h y d r o c e p h a l u s has b e c o m e r a r e b e c a u s e of the risk o f c h r o n i c t h r o m b o - e m b o l i s m a n d s u b s e q u e n t p u l m o n a r y h y p e r t e n s i o n a s s o c i a t e d with an u n f a v o u r a b l e prognosis. H o w e v e r , t h e r e r e m a i n s a g r o u p of p a t i e n t s in w h o m v e n t i c u l o - a t r i a l shunts h a v e b e e n ins e r t e d in t h e past. In this g r o u p , c o m p l i c a t i o n s can occur after m a n y years. W e r e p o r t o n a 1 3 - y e a r - o l d girl with pulmonary hypertension caused by chronic thrombo-emb o l i s m f r o m a v e n t i c u l o - a t r i a l shunt. Key words: H y d r o c e p h a l u s - V e n t r i c u l o - a t r i a l shunt Pulmonary hypertension
Introduction N o w a d a y s , v e n t i r c u l o p e r t i o n e a l shunts are p e r f o r m e d m o r e f r e q u e n t l y t h a n v e n t r i c u l o a t r i a l shunts in t r e a t i n g p r o g r e s s i v e h y d r o c e p h a l u s in children. I n p a r t , this is a c o n s e q u e n c e of t h r o m b o - e m b o l i c c o m p l i c a t i o n s which occur f r e q u e n t l y in this t y p e of c a t h e t e r [2, 3, 8]. T h e o v e r a l l i n c i d e n c e of t h r o m b o - e m b o l i s m m a y b e as high as 55% if r o u t i n e m i c r o s c o p i c lung sections a r e r e v i e w e d [5]. T h e s e c o m p l i c a t i o n s a r e d u e e i t h e r to e m b o l i disl o d g i n g f r o m the e n d of t h e c a t h e t e r , f r e q u e n t l y r e l a t e d to c h r o n i c shunt infection, or to t h e i n t r o d u c t i o n of b r a i n t h r o m b o p l a s t i n f r o m t h e C S F into t h e c i r c u l a t i o n , resulting in t h r o m b o s i s at t h e p o i n t of c o n t a c t with p l a s m a c o a g u l a t i o n factors [1, 4]. H o w e v e r , t h e r e is still a g r o u p of p a t i e n t s in w h o m v e n t r i c u l o - a t r i a l shunts h a v e b e e n i n s e r t e d in t h e past. I n this g r o u p , c o m p l i c a t i o n s can occur e v e n after m a n y y e a r s , as i l l u s t r a t e d b y t h e following case r e p o r t .
Case report A 13-year-old girl was admitted for operative correction of a progressive thoracolumbar scoliosis. On the 2nd day of life a lumbosacral meningomyelocele had been surgically corrected. A ventriculo-atrial shunt was inserted at 4 months of age for progressive Correspondence to: K. Haasnoot
hydrocephalus. Postoperatively, she developed a ventriculitis for which an extended course of antibiotics was given. The shunt was revised twice because of increased intracranial pressure. She was discharged at 6 months of age in a satisfactory condition. In the last few years, a scoliosis developed which caused discomfort and pain and for which operative correction was necessary. Preoperatively, the patient presented with a history of fatigue, progressive dyspnoea and ankle oedema. On examination she was moderately mentally retarded and paraplegic. The venticulo-atrial shunt was functioning well and there were no signs of raised intracranial pressure. She was afebrile and in no respiratory distress, although there was a productive cough. Auscultation showed no pulmonary abnormalities, normal heart sounds and a soft diastolic murmur at the left sternal border, compatible with pulmonary insufficiency. The liver was not enlarged. Routine examination of the blood showed no abnormalities. The chest X-ray film showed no cardiac enlargement, the tip of the venticulo-atrial shunt was visible in the superior vena cava. The electrocardiogram suggested right atrial and right ventricular hypertrophy. Echocardiography showed an enlarged and hypertrophied right ventricle, and pulmonary artery pressure appeared to be elevated on estimation by Doppler. The suspected pulmonary hypertension was confirmed by pulmonary arterial catherisation, showing a mean pulmonary arterial pressure of 70 mm Hg. Subsequently the mean pulmonary arterial pressure, monitored with a SwanGanz cathether over a 48 h period both with and without extra oxygen, varied between 30 and 85 mm Hg. Normally the mean pulmonary arterial pressure is below 20 mm Hg. The mean pulmonary wedge pressure was normal (values between 10 and 18 mm Hg). In view of the pulmonary hypertension further investigations were conducted to elucidate the cause and the planned operation on the scoliosis was postponed. There were three possible causes of the pulmonary hypertension: in the first place pulmonary hypertension resulting from chronic thrombo-embolism from the ventriculo-atrial shunt or from thrombogenic brain thromboplastin released into the pulmonary circulation via the shunt catheter; secondly idiopathic pulmonary hypertension and finally posthypoxic pulmonary hypertension. No hypoxic periods were recorded using transcutaneous pulse-oximetry during sleep which showed that posthypoxic pulmonary hypertension was unlikely. A radionuclide ventilation-perfusion scan demonstrated multiple filling defects in both lung fields consistent with pulmonary emboli. Subsequently a lung-biopsy was performed, revealing the microscopic picture of post-thrombotic arteriopathy (Fig. 1). Finally, duplex scanning of the inferior vena cava, iliac and femoral veins showed no venous thrombosis in these blood vessels. These findings confirmed the diagnosis of pulmonary hypertension resulting from chronic thrombo-embolism from the venticulo-atrial shunt or from brain thromboplastin released into the pulmonary circulation.
749
Fig.la, b. Lung biopsy section (elastic tissue stain, magnification • 170) (a) eccentric intimal fibrosis and (b) an intravascular septum in a pulmonary artery branch a
b
It was decided to remove the ventriculo-atrial shunt in order to eliminate the source of further pulmonary embolism. The creation of a ventriculoperitoneal shunt was also necessary to control intracranial pressure. No complications occurred during and after this operation and the patient was discharged after a few days. Eight days later the patient suddenly died at home. Post mortem examination was not performed.
Discussion
Pulmonary hypertension as a consequence of chronic pulmonary embolism is seldom considered in the paediatric patient. A review of the literature of the past 30 years yielded only 25 recorded cases [3, 7, 9, 11]. Of these 25 patients, 22 had emboli as a complication of a ventriculo-atrial shunt, and one had emboli secondary to indwelling venous hyperalimentation. Two cases were not catheter-related [11]. Although in recent years there has been a preference for the peritoneal cavity over the atrium for CSF diversion, there remain patients in whom ventriculo-atrial catheters have been inserted in the past. In this group, pulmonary hypertension and cor pulmonale due to chronic thrombo-embolism can occur after many years. It is associated with an unfavourable prognosis, despite removal of the offending catheter [8]. Detecting a patient with developing pulmonary hypertension is difficult. Early clinical findings may be subtle and are not often appreciated. When the condition is advanced, the patient usually presents with a history of fatigue, coughing (sometimes haemoptysis), chest pain, dyspnoea, ankle oedema and febrile episodes. Physical examination shows a raised jugular venous pressure with enlargement of the liver and "pitting" oedema of ankles and feet. Auscultation of the heart may reveal an accentuated single second heart sound and a soft diastolic
murmur at the left sternal border due to pulmonary valve insufficiency. In the chest roentgenogram pulmonary hypertension is indicated by cardiomegaly, large central pulmonary arteries with rapid distal tapering and reduced perfusion (oligaemia) [11]. An electrocardiogram will demonstrate right ventricular and atrial hypertrophy (high voltages, right-axis deviation and P-pulmonale). A radionuclide ventilation-perfusion scan will show multiple perfusion defects caused by chronic micro-embolism. Pulmonary hypertension can be confirmed by pulmonary artery catheterisation, demonstrating an elevated mean pulmonary artery pressure and a normal pulmonary artery wedge pressure. In order to elucidate the cause of pulmonary hypertension, a lung biopsy should be performed. Microscopically, the arterioles will show media hypertrophy, intima proliferation and, more specific in chronic pulmonary thrombo-embolism, eccentric intimal fibrosis (Fig. l a) and occlusion of branches of the pulmonary artery by emboli with recanalisation and formation of intravascutar septa (Fig. lb). The presence of a foreign body in the cardiovascular system for long periods of time may result in thromboemboli. Thrombi may break off and lodge in the pulmonary vascular tree; over a period of time this seeding can produce pulmonary hypertension and chronic cor pulmonale. In the future this complication will occur less frequently in CSF diversion owing to the more frequent use of ventriculoperitoneal in place of ventriculo-atrial shunts. However, the same complications arise from central venous catheters used for total parenteral nutrition and long-term chemoterapy [6, 10, 11]. Consequently, physicians should be alert to the possibility of pulmonary hypertension in children with prolonged indwelling catheters.
750
Acknowledgements. We thank Prof. Dr. J. Huber, Department of Pathology, University of Utrecht, the Netherlands, for the microscopic data and Dr. R. Feldman, University Children's Hospital "Het Wilhelmina Kinderziekenhuis", Utrecht, The Netherlands, for his help in preparing the manuscript.
References 1. Anderson FM (1973) Ventriculoatrial shunts. Identification and control of practical problems in 143 cases. J Pediatr 82: 222-227 2. Buck JR, Connors RH, Coon WW, Weintraub WH, Wesley JR, Coran AG (t981) Pulmonary embolism in children. J Pediatr Surg 16: 385-391 3. Butler PJ, Wheeler RA, Spargo PM (1990) Life-threatening complications during anaesthesia in a patient with a ventriculoatrial shunt and pulmonary hypertension. Anaesthesia 45: 946-948 4. Favara BE, Paul PN (1967) Thromboembolism and cor pulmonale complicating ventriculovenous shunts. J Am Med Ass 199 : 688-671
5. Friedman S, Zita-Gozum C, Chatten J (1964) Pulmonary vascular changes complicating ventriculovascular shunting for hydrocephalus. J Pediatr 64 : 305-314 6. Haber SL, Bennington JL (1962) Pulmonary embolism in an infant. J Pediatr 61 : 759-762 7. Normand J, Gilly R, Loire R, Lapras C, Bozio A, Gidrol C (1976) Coeur pulmonaire chronique au cours des derivations ventriculo-atriales chez l'enfant. Arch Mal Coeur 12:12931296 8. Piat JH, Hoffman HJ (1989) Cor pulmonale: a lethal complication of ventriculoatrial CSF diversion. Childs Nerv Syst 5 : 2931 9. Pryds OA, Petersen SA, Jacobsen JR, Gjerris F (1989) Pulmonary hypertension in children treated with ventriculoatrial shunt in hydrocephalus. Ugeskr Laeger 151 : 570-572 10. Rockoff MA, Gang DL, Vacanti JP (1984) Fatal pulmonary embolism following removal of a central venous cathether. J Pediatr Surg 19 : 307-309 11. Woodruff WW, Merten DF, Wagner ML, Kirks DR (1986) Chronic pulmonary embolism in children. Radiology 159:511514
European Journal of
Pediatrics
9 Springer-Verlag 1992
Pulmonary hypertension complicating a ventriculo-atrial shunt K. H a a s n o o t 1 and A. J, van Vught 2 1Paediatric Intensive Care Unit, Free University Hospital, P.O. Box 7057, NL-1007 MB Amsterdam, The Netherlands 2Paediatric Intensive Care Unit, University Children's Hospital "Het Wilhelmina Kinderziehkenhuis", Utrecht, The Netherlands Received July 22, 1991 / Accepted after revision February 16, 1992
Abstract. T h e use of v e n t r i c u l o - a t r i a l shunts for C S F div e r s i o n in d e v e l o p i n g h y d r o c e p h a l u s has b e c o m e r a r e b e c a u s e of the risk o f c h r o n i c t h r o m b o - e m b o l i s m a n d s u b s e q u e n t p u l m o n a r y h y p e r t e n s i o n a s s o c i a t e d with an u n f a v o u r a b l e prognosis. H o w e v e r , t h e r e r e m a i n s a g r o u p of p a t i e n t s in w h o m v e n t i c u l o - a t r i a l shunts h a v e b e e n ins e r t e d in t h e past. In this g r o u p , c o m p l i c a t i o n s can occur after m a n y years. W e r e p o r t o n a 1 3 - y e a r - o l d girl with pulmonary hypertension caused by chronic thrombo-emb o l i s m f r o m a v e n t i c u l o - a t r i a l shunt. Key words: H y d r o c e p h a l u s - V e n t r i c u l o - a t r i a l shunt Pulmonary hypertension
Introduction N o w a d a y s , v e n t i r c u l o p e r t i o n e a l shunts are p e r f o r m e d m o r e f r e q u e n t l y t h a n v e n t r i c u l o a t r i a l shunts in t r e a t i n g p r o g r e s s i v e h y d r o c e p h a l u s in children. I n p a r t , this is a c o n s e q u e n c e of t h r o m b o - e m b o l i c c o m p l i c a t i o n s which occur f r e q u e n t l y in this t y p e of c a t h e t e r [2, 3, 8]. T h e o v e r a l l i n c i d e n c e of t h r o m b o - e m b o l i s m m a y b e as high as 55% if r o u t i n e m i c r o s c o p i c lung sections a r e r e v i e w e d [5]. T h e s e c o m p l i c a t i o n s a r e d u e e i t h e r to e m b o l i disl o d g i n g f r o m the e n d of t h e c a t h e t e r , f r e q u e n t l y r e l a t e d to c h r o n i c shunt infection, or to t h e i n t r o d u c t i o n of b r a i n t h r o m b o p l a s t i n f r o m t h e C S F into t h e c i r c u l a t i o n , resulting in t h r o m b o s i s at t h e p o i n t of c o n t a c t with p l a s m a c o a g u l a t i o n factors [1, 4]. H o w e v e r , t h e r e is still a g r o u p of p a t i e n t s in w h o m v e n t r i c u l o - a t r i a l shunts h a v e b e e n i n s e r t e d in t h e past. I n this g r o u p , c o m p l i c a t i o n s can occur e v e n after m a n y y e a r s , as i l l u s t r a t e d b y t h e following case r e p o r t .
Case report A 13-year-old girl was admitted for operative correction of a progressive thoracolumbar scoliosis. On the 2nd day of life a lumbosacral meningomyelocele had been surgically corrected. A ventriculo-atrial shunt was inserted at 4 months of age for progressive Correspondence to: K. Haasnoot
hydrocephalus. Postoperatively, she developed a ventriculitis for which an extended course of antibiotics was given. The shunt was revised twice because of increased intracranial pressure. She was discharged at 6 months of age in a satisfactory condition. In the last few years, a scoliosis developed which caused discomfort and pain and for which operative correction was necessary. Preoperatively, the patient presented with a history of fatigue, progressive dyspnoea and ankle oedema. On examination she was moderately mentally retarded and paraplegic. The venticulo-atrial shunt was functioning well and there were no signs of raised intracranial pressure. She was afebrile and in no respiratory distress, although there was a productive cough. Auscultation showed no pulmonary abnormalities, normal heart sounds and a soft diastolic murmur at the left sternal border, compatible with pulmonary insufficiency. The liver was not enlarged. Routine examination of the blood showed no abnormalities. The chest X-ray film showed no cardiac enlargement, the tip of the venticulo-atrial shunt was visible in the superior vena cava. The electrocardiogram suggested right atrial and right ventricular hypertrophy. Echocardiography showed an enlarged and hypertrophied right ventricle, and pulmonary artery pressure appeared to be elevated on estimation by Doppler. The suspected pulmonary hypertension was confirmed by pulmonary arterial catherisation, showing a mean pulmonary arterial pressure of 70 mm Hg. Subsequently the mean pulmonary arterial pressure, monitored with a SwanGanz cathether over a 48 h period both with and without extra oxygen, varied between 30 and 85 mm Hg. Normally the mean pulmonary arterial pressure is below 20 mm Hg. The mean pulmonary wedge pressure was normal (values between 10 and 18 mm Hg). In view of the pulmonary hypertension further investigations were conducted to elucidate the cause and the planned operation on the scoliosis was postponed. There were three possible causes of the pulmonary hypertension: in the first place pulmonary hypertension resulting from chronic thrombo-embolism from the ventriculo-atrial shunt or from thrombogenic brain thromboplastin released into the pulmonary circulation via the shunt catheter; secondly idiopathic pulmonary hypertension and finally posthypoxic pulmonary hypertension. No hypoxic periods were recorded using transcutaneous pulse-oximetry during sleep which showed that posthypoxic pulmonary hypertension was unlikely. A radionuclide ventilation-perfusion scan demonstrated multiple filling defects in both lung fields consistent with pulmonary emboli. Subsequently a lung-biopsy was performed, revealing the microscopic picture of post-thrombotic arteriopathy (Fig. 1). Finally, duplex scanning of the inferior vena cava, iliac and femoral veins showed no venous thrombosis in these blood vessels. These findings confirmed the diagnosis of pulmonary hypertension resulting from chronic thrombo-embolism from the venticulo-atrial shunt or from brain thromboplastin released into the pulmonary circulation.
749
Fig.la, b. Lung biopsy section (elastic tissue stain, magnification • 170) (a) eccentric intimal fibrosis and (b) an intravascular septum in a pulmonary artery branch a
b
It was decided to remove the ventriculo-atrial shunt in order to eliminate the source of further pulmonary embolism. The creation of a ventriculoperitoneal shunt was also necessary to control intracranial pressure. No complications occurred during and after this operation and the patient was discharged after a few days. Eight days later the patient suddenly died at home. Post mortem examination was not performed.
Discussion
Pulmonary hypertension as a consequence of chronic pulmonary embolism is seldom considered in the paediatric patient. A review of the literature of the past 30 years yielded only 25 recorded cases [3, 7, 9, 11]. Of these 25 patients, 22 had emboli as a complication of a ventriculo-atrial shunt, and one had emboli secondary to indwelling venous hyperalimentation. Two cases were not catheter-related [11]. Although in recent years there has been a preference for the peritoneal cavity over the atrium for CSF diversion, there remain patients in whom ventriculo-atrial catheters have been inserted in the past. In this group, pulmonary hypertension and cor pulmonale due to chronic thrombo-embolism can occur after many years. It is associated with an unfavourable prognosis, despite removal of the offending catheter [8]. Detecting a patient with developing pulmonary hypertension is difficult. Early clinical findings may be subtle and are not often appreciated. When the condition is advanced, the patient usually presents with a history of fatigue, coughing (sometimes haemoptysis), chest pain, dyspnoea, ankle oedema and febrile episodes. Physical examination shows a raised jugular venous pressure with enlargement of the liver and "pitting" oedema of ankles and feet. Auscultation of the heart may reveal an accentuated single second heart sound and a soft diastolic
murmur at the left sternal border due to pulmonary valve insufficiency. In the chest roentgenogram pulmonary hypertension is indicated by cardiomegaly, large central pulmonary arteries with rapid distal tapering and reduced perfusion (oligaemia) [11]. An electrocardiogram will demonstrate right ventricular and atrial hypertrophy (high voltages, right-axis deviation and P-pulmonale). A radionuclide ventilation-perfusion scan will show multiple perfusion defects caused by chronic micro-embolism. Pulmonary hypertension can be confirmed by pulmonary artery catheterisation, demonstrating an elevated mean pulmonary artery pressure and a normal pulmonary artery wedge pressure. In order to elucidate the cause of pulmonary hypertension, a lung biopsy should be performed. Microscopically, the arterioles will show media hypertrophy, intima proliferation and, more specific in chronic pulmonary thrombo-embolism, eccentric intimal fibrosis (Fig. l a) and occlusion of branches of the pulmonary artery by emboli with recanalisation and formation of intravascutar septa (Fig. lb). The presence of a foreign body in the cardiovascular system for long periods of time may result in thromboemboli. Thrombi may break off and lodge in the pulmonary vascular tree; over a period of time this seeding can produce pulmonary hypertension and chronic cor pulmonale. In the future this complication will occur less frequently in CSF diversion owing to the more frequent use of ventriculoperitoneal in place of ventriculo-atrial shunts. However, the same complications arise from central venous catheters used for total parenteral nutrition and long-term chemoterapy [6, 10, 11]. Consequently, physicians should be alert to the possibility of pulmonary hypertension in children with prolonged indwelling catheters.
750
Acknowledgements. We thank Prof. Dr. J. Huber, Department of Pathology, University of Utrecht, the Netherlands, for the microscopic data and Dr. R. Feldman, University Children's Hospital "Het Wilhelmina Kinderziekenhuis", Utrecht, The Netherlands, for his help in preparing the manuscript.
References 1. Anderson FM (1973) Ventriculoatrial shunts. Identification and control of practical problems in 143 cases. J Pediatr 82: 222-227 2. Buck JR, Connors RH, Coon WW, Weintraub WH, Wesley JR, Coran AG (t981) Pulmonary embolism in children. J Pediatr Surg 16: 385-391 3. Butler PJ, Wheeler RA, Spargo PM (1990) Life-threatening complications during anaesthesia in a patient with a ventriculoatrial shunt and pulmonary hypertension. Anaesthesia 45: 946-948 4. Favara BE, Paul PN (1967) Thromboembolism and cor pulmonale complicating ventriculovenous shunts. J Am Med Ass 199 : 688-671
5. Friedman S, Zita-Gozum C, Chatten J (1964) Pulmonary vascular changes complicating ventriculovascular shunting for hydrocephalus. J Pediatr 64 : 305-314 6. Haber SL, Bennington JL (1962) Pulmonary embolism in an infant. J Pediatr 61 : 759-762 7. Normand J, Gilly R, Loire R, Lapras C, Bozio A, Gidrol C (1976) Coeur pulmonaire chronique au cours des derivations ventriculo-atriales chez l'enfant. Arch Mal Coeur 12:12931296 8. Piat JH, Hoffman HJ (1989) Cor pulmonale: a lethal complication of ventriculoatrial CSF diversion. Childs Nerv Syst 5 : 2931 9. Pryds OA, Petersen SA, Jacobsen JR, Gjerris F (1989) Pulmonary hypertension in children treated with ventriculoatrial shunt in hydrocephalus. Ugeskr Laeger 151 : 570-572 10. Rockoff MA, Gang DL, Vacanti JP (1984) Fatal pulmonary embolism following removal of a central venous cathether. J Pediatr Surg 19 : 307-309 11. Woodruff WW, Merten DF, Wagner ML, Kirks DR (1986) Chronic pulmonary embolism in children. Radiology 159:511514
