British Heart_Journal, I970, 32, 269.

Pulmonary hypertension due tomicro-thromboembolism from splenic andportal veins after portacaval anastomosis

M. Sallam and W. C. WatsonFrom the Gastro-intestinal Unit, Glasgow Royal Infirmary

This is a presentation of a unique case of pulmonary hypertension in a x5-year-old girl, due tomicro-thromboembolism from splenoportal veins through a portacaval anastomosis.

Micro-thromboembolism producing insidiouspulmonary hypertension is an importantcategory of pulmonary embolism. It contrastswith other types which, because the emboliare larger, present with acute or subacutesymptoms. Wood (1956) in his descriptionof subacute thromboembolic pulmonaryhypertension stressed the importance of earlydiagnosis and treatment with anticoagulants.Goodwin (I960) pointed out that thrombo-embolism was probably the commonest causeof obliterative pulmonary vascular disease.He and his colleagues (Goodwin, Harrison,and Wilcken, I963) classified patients withchronic pulmonary hypertension of embolicorigin into two groups, those with largeemboli affecting main or segmental arteries,and others with small emboli affecting micro-scopic vessels leading to progressive oblitera-tive thromboembolic pulmonary hyperten-sion. A more recent review (Fleischner, I967)separates the groups on a clinical as well asradiological basis into those with an acuteonset of chest pain, dyspnoea, and haemop-tysis, and those with insidious onset of pul-monary hypertension who are likely to bediagnosed as idiopathic pulmonary hyper-tension.The source of pulmonary embolism is

thrombus which is usually in the leg veins,occasionally in the pelvic veins, and rarely inthe heart in conditions such as endocardialfibrosis or fibroelastosis, or after myocardialinfarction affecting the right ventricle. Bed-rest, immobilization, trauma to legs or pelvis,malignancy, oral contraceptives, heart failure,and atrial fibrillation are important factors.The incidence is increased after the age of 20.

It is doubtful if age by itself is an importantfactor in the aetiology of thromboembolism(Marshall, I965), though the incidence is par-ticularly low in children.

Case reportThis is an account of a unique case of pulmonaryhypertension secondary to thromboembolismarising in the portal and splenic veins of a I5-year-old girl, after a portacaval anastomosis.The patient presented at the age of 5 with

haematemesis and melaena from oesophagealvarices. She had hepatosplenomegaly, and thediagnosis of Banti's syndrome was made. Shecontinued to have episodes of acute alimentarybleeding, until at the age of 9 a portacavalanastomosis was performed. At operation thedistal half of the portal vein was seen to be vari-cosed and thrombosed, and there was some looseclot. Liver biopsy showed no evidence ofcirrhosis.After operation there was considerable involutionof the spleen and the oesophageal varices dis-appeared. There was no further trouble withanaemia or bleeding and her growth and develop-ment proceeded normally.At a review medical examination at the age of

I5 there were clinical signs of pulmonary hyper-tension and on direct questioning the patientadmitted to increasing breathlessness over aperiod of I0 months, without cough, chest pain,or haemoptysis. A provisional diagnosis ofthromboembolic pulmonary hypertension wasmade and she was admitted for investigation.

She was a well-developed girl without cya-nosis, pallor, or finger clubbing. The externaljugular vein filled to 5 cm. above the angle ofthe sternum, and there was a prominent A wave.The pulse was regular at 68/min. BP I30/70mm. Hg. The apex beat was in the sth interspacein the midclavicular line. There was a right ventri-cular heave over the left parasternal area. The

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270 Sallam and Watson

first sound at the pulmonary area was normaland followed by an ejection systolic murmurgrade 3/6. The second sound at the same areawas narrowly split, with accentuation of thepulmonary component.The liver and spleen were enlarged 3-4 cm.

and were firm. The chest and central nervoussystem were normal.

Investigations Hb I3-8 g./Ioo ml.; WBC3800/cu. mm.; platelets io8,ooo/cu. mm.;reticulocytes 5%; urea, electrolytes, standardliver function tests, and coagulation screen wereall normal. Electrocardiogram showed moderateright ventricular hypertrophy. Chest x-rayshowed slight enlargement of the heart, withprominence of the pulmonary artery. Pulmonaryfunction tests were normal and, in particular,there was no evidence of alveo-capillary block.

Cardiac catheterization and pulmonary angio-graphy were performed (Fig. i). The pulmonaryartery pressure was 70/32 mm. Hg, with a meanpressure of 48 mm. Hg. There was no oblitera-tion of major arteries or evidence of a left-sidedheart lesion or left-to-right shunt.A splenic venogram (Fig. 2) showed the porto-

caval anastomosis to be widely patent. Pressurein the splenic and portal veins was normal, buta movable small mass, probably thrombus, wasobserved in the veins at the hilum of the spleen.

DiscussionThis case represents the very rare situationin which embolus arising in the portal veincould reach the lung through a portacavalanastomosis and lead to obstructive pulmon-ary hypertension. The diagnosis was madeinitially on the positive factors compatiblewith it and corroborated by exclusion ofother causes of pulmonary hypertension.Post-capillary (passive) hypertension second-ary to left-sided heart lesions such as mitralstenosis, mitral incompetence, and atrialmyxoma as well as stenosis of the pulmonaryveins was excluded by angiography. Theso-called high output states of anaemia andthyrotoxicosis were also absent, and evenliver cirrhosis was excluded by liver biopsy.No parenchymal lung disease could be shownclinically, radiologically, or by pulmonaryfunction tests. There were no features tosuggest those rare types of pulmonary vas-cular disease found in association with sys-temic lupus erythematosus or periarteritisnodosa. We are aware that cardiac output isincreased after portacaval anastomosis inhuman patients (Even et al., I965) as wellas after experimental surgery in dogs (John-son and Lambert, I967). This increase byitself is probably insufficient to cause a mod-erately severe pulmonary hypertension. Thediagnosis of primary or idiopathic pulmonary

FIG . I Pulmonary angiogram showing en-larged main pulmonary artery (MPA). Thereis some diminution in the extension of thesmall vessels, particularly at both lowerzones.

hypertension is made with diminishing fre-quency as other causes of pulmonary hyper-tension are discovered (Friedberg, I966).

In the patient reported here the site oforigin of the pulmonary emboli was acceptedas being in the portal and splenic veins andtheir tributaries. Evidence supporting thisconclusion was the operation report of portalvein pathology manifest as tortuosity andthrombosis, plus the recent venogram showingthrombus at the hilum of the spleen.

Treatment and prognosis Anticoagulantsare indicated as early as possible in patientswith recurrent and subacute thrombo-embolism, and before obliterative lesions inthe pulmonary vasculature are well estab-lished. Wood (1956) regarded anticoagulanttherapy as worth while, especially if givenearly in the course of the disease. Thompsonand Hamilton (I962) and Goodwin et al.(I963) were, on the other hand, less enthusi-

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Pulmonary hypertension due to micro-thromboembolism after portacaval anastomosis 271

astic about anticoagulant therapy, since intheir experience progressive obliterative vas-cular disease eventually led to right heartfailure in both treated and untreated patients,though there was a slightly better prognosisin the treated cases.

It seems a reasonable premise neverthelessthat better results should be expected if anti-coagulants are given before well-establishedobliterative pulmonary hypertension hasoccurred.

Splenectomy was not advised in this patientat the present time for two reasons. First,the mild degree of hypersplenism presentwas not sufficient to warrant splenectomy,with its possible sequel of an increase inplatelet count. Secondly the splenic veinwas probably not the only source ofembolism;the portal vein and its tributaries were alsolikely contributors.One aspect of this case is puzzling and that

is why in the course of six years' exposureto the risk of pulmonary embolism thispatient never had at least one embolismlarge enough to cause overt symptoms. Thepossible answer may be that chronic thrombo-cytopenia has meant that whatever thrombuswas produced in the splenoportal veins wasof poor quality and readily broken intomicroemboli.

We are indebted to Dr. Moran of the Cardio-respiratory Unit and Drs. Moule and McKellarof the Department of Radiology for the specialinvestigations.

ReferencesEven, P., Nicollo, F., Benhamou, J. P., and Fauvert,

R. (i965). Le debit cardiaque au cours des mal-adies du foie. Effets de l'anastomose porto-caveet des diuretiques. Revue Francaise d'.tudes Clini-ques et Biologiques, 10, 799.

Fleischner, F. G. (I967). Recurrent pulmonary em-bolism and cor pulmonale. New England Journalof Medicine, 276, 1213.

Friedberg, C. K. (I966). Diseases of the Heart, 3rd ed.Saunders, Philadelphia.

FIG. 2 Splenogram showing a filling defectin the splenic vein close to the hilum suggest-ing a thrombus (upper arrow). The lowerarrows show further filling defects in a veinat the lower pole of the spleen. These areprobably also thrombi. SV, splenic vein;PV, portal vein; IHR, varicose intrahepaticportal radicles.

Goodwin, J. F. (I960). The nature of pulmonaryhypertension. In Clinical Disorders of the PulmonaryCirculation, p. 57. Ed. by R. Daley, J. F. Good-win, and R. E. Steiner. Churchill, London.

, Harrison, C. V., and Wilcken, D. E. L. (I963)Obliterative pulmonary hypertension and thrombo-embolism. British MedicalJournal, I, 70I and 777.

Johnson, G., and Lambert, J. (I967). Cardiac outputafter portacaval shunt. Annals of Surgery, x66,207.

Marshall, R. (I965). Pulmonary Embolism-Mechanismand Management. Thomas, Springfield, Illinois.

Thompson, E. N., and Hamilton, M. (I962). Pul-monary-embolic disease. Lancet, I, I369.

Wood, P. (1956). Diseases of the Heart and Circulation,2nd ed. Eyre and Spottiswoode, London.

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