Cor Pulmonale as a Complication of Ventriculo‐atrial Shunts Reviewed

Abstract

SUMMARY The authors present the case of a nine‐year‐old patient with spina bifida and hydrocephalus treated with a ventriculo‐atrial shunt who developed fatal pulmonary hypertension 5 1/2 years after his last valve revision; he had been well and active up to one month before his death. Details of the dramatic cardiovascular findings are given. The problems of treatment, once symptoms occur, and the difficulty of pre‐symptomatic detection are discussed. RÉSUMÉ Coeur pulmonaire compliquant une dérivation ventriculoatriale Les auteurs rapportent le cas d'un patient de neuf ans présentant un spina bifida et une hydrocéphalie traitée par une dérivation ventriculoatriale qui développa une hypertension pulmonaire mortelle cinq ans et demi après la dermière révision de valve; il fut extrěment bien et actif jusqu'au mois précédent le décès. Les détails des données cardio‐vasculaires dramatiques sont précisés. Les problèmes de traitement, une fois les symptomes apparus et la difficultée de la détection pré‐symptomatique sont discutés. ZUSAMMENFASSUNG 'Cor pulmonale' als Komplikotion bei einem ventrikulo‐avialen shunt Die Autoren stellen den Fall eines neun‐jährigen Patienten mit Spina bifida und Hydrozephalus und ventrikulo‐atrialem Shunt vor, der 5 ½ Jahre nach der letzten Ventilrevision eine zum Tode fiihrende pulmonale Hypertension entwickelte; er war bis zu einem Monat vor seinem Tod sehr gesund und aktiv. Es werden Einzelheiten der dramatischen kardio‐vaskulären Befunde aufgeführt. Die Probleme der Behandlung—wenn Symptome auftreten—und die Schwierigkeiten einer prä‐symptomatischen Diagnose werden diskutiert. RESUMEN 'Cor pulmonale ' como complicación de derivaciones ventriculo‐otriales. Revisión Los autores presentant el caso de una paciente de nueve años con espína bifida e hidrocefalia, tratada con una derivación ventriculo‐atrial que padeció una hipertension pulmonar fatal 5 anAos y medio después de la última revision de su válvula. Estaba extremadamente bien y activa hasta un mes antes de su muerte. Se dan detalles de los marcados hallazgos cardiovasculares. Se discuten los problemas de tratamiento una vez aparecidos los sintomas y la dificultad de una detección.

Pulmonary hypertension after ventriculoatrial shunt implantation

“…Perfusion defects were found in 3 asymptomatic children, and it was concluded that the perfusion scan is a safe and simple procedure in patients with shunts. Finally, Sleigh et al 17 prospectively studied 20 asymptomatic patients (mean age 9 years) with VA shunts by clinical examination, electrocardiography, chest x-ray, and echocardiography, and found no evidence of pulmonary hypertension.…”

Thrombosis associated with ventriculoatrial shunts

“…The favorable intervention lead to notable concerns with the recognition of various range of severe and even life-threatening complications due to the specificity of cardiac placement and systemic drainage [1,8,16]. Although all ventricular shunts are susceptible to malfunction due to obstruction, disconnection, and infection, patients with VAS are at risk for different complications than those with other types of shunts, the most unique of which are cardiopulmonary complications, such as atrial thrombi, PE, PH, cor pulmonale [2,[4][5][6]8]. Conversely, the complications associated with VPS are potentially less morbid and are more easily manageable.…”

“…The rate of thromboembolic events in VAS cannot be fully explained on the basis of a foreign body in the right atrium, as patients with pacemaker leads do not have equivalent rates of thromboembolism [ 23 ]. It has been suggested that chronic PE leading to PH and death is not due simply to the production of showers of thrombo-emboli, but probably to a reaction of the pulmonary endothelium to some contents of CSF, leading to in situ thrombosis [ 5 , 10 , 24 ]. Some in vitro studies showed that adding CSF to blood makes blood hypercoagulable.…”

“…When the condition is advanced, the patient usually presents with a history of fatigue, coughing (sometimes haemoptysis), chest pain, dyspnea, and febrile episodes [ 3 , 10 , 14 , 32 ]. Once symptomatic, PH tends to be relentlessly progressive, developing cor pulmonale and leading to death [ 5 ]. Therefore, it has been suggested that regular monitoring for symptoms and signs of cardiopulmonary disease is mandatory, including shortness of breath, dyspnea on exertion, failure to thrive, jugular venous distension, cyanosis, precordial heaves, cardiomegaly, and peripheral edema.…”

“…The most common alternative is the ventriculoatrial shunt (VAS) to the right atrium [2]. However, various complications associated with a VAS have gradually been reported in the literature, more specific of them are cardiopulmonary complications which are potentially lifethreatening, including atrial or venous thrombosis with associated pulmonary embolism (PE), pulmonary hypertension (PH), and cor pulmonale [2][3][4][5][6][7][8]. What's worse, only a small proportion of patients with thromboembolism have the perceptible features associated with short-term clinical deterioration while most of VAS recipients may have a long asymptomatic latency after shunt insertion, which may result in misdiagnosis or delayed diagnosis [3,4,9,10].…”