Pulmonary Hypertension as a First Presentation for Schistosomiasis: a Case Report of an Unusual Presentation

Case Report

Austin J Clin Case Rep. 2019; 6(1): 1140.

Pulmonary Hypertension as a First Presentation for Schistosomiasis: a Case Report of an Unusual Presentation

Mogahed Ismail Hassan Hussein*

Department of Physiology University of Gezira, Division of Internal Medicine, Wad Medani Hospital, Sudan

*Corresponding author: Mogahed Ismail Hassan Hussein, Department of Physiology University of Gezira, Division of Internal Medicine, Wad Medani Hospital, Sudan

Received: January 21, 2019; Accepted: February 20, 2019; Published: February 27, 2019


Introduction: The combination of schistosomiasis and pulmonary hypertension was always recognized as a rare one, to the point that studies used to consider pulmonary hypertension as a manifestation of hepatosplenic schistosomiasis but none of them has considered it as a manifestation of Schistosoma infection until recently. In a case series study, 18.5% of patients that have a documented hepatosplenic schistosomiasis were found with pulmonary hypertension. Schistosomiasis rarely causes pulmonary hypertension without evident hepatosplenic manifestations. Here we are reporting a case of a patient whose first clinical presentation was features of pulmonary hypertension. The patient developed SOB and subsequently went on to develop clinical manifestations of the disease, paradoxically without ascites, splenomegaly or any features of the hepatosplenic disease.

Discussion: We use this case as an opportunity to outline pathological mechanisms, causes, and classification of pulmonary hypertension. We also review the literature regarding pulmonary hypertension as a first presentation for schistosomiasis to establish the rarity of this case.

Conclusion: A structured and thorough workup for pulmonary hypertension is emphasized. It is important to exclude all other secondary causes to be able to diagnose primary pulmonary hypertension especially in the absence of a positive family history and advanced diagnostic technology.

Keywords: Schistosomiasis; Pulmonary hypertension; Periportal fibrosis


APAH: Associated Pulmonary Arterial Hypertension; ECG: Electrocardiogram; FPAH: Familial Pulmonary Arterial Hypertension; IPAH: Idiopathic Pulmonary Arterial Hypertension; LPSH: Left Parasternal Heave; NYHA: New York Heart Association; PAH: Pulmonary Arterial Hypertension; PAP: Pulmonary Arterial Pressure; PMH: Past Medical History; PVR: Pulmonary Vascular Resistance; RV: Right Ventricle; SMCs: Smooth Muscle Cells; SOB: Shortness of Breath; WHO: World Health Organization


The blood is pumped from the right ventricle through the lungs and then to the left atrium, which leads to the systemic circulation, this circuit constitute the pulmonary circulation.

The blood from the right ventricle flow through the main pulmonary artery, which then divides into the two pulmonary arteries, one of the supplies the right lung and the other supplies the left. In the lungs, those arteries branch excessively and are connected to arterioles, they lead to capillaries that unite into venules and then veins. Four pulmonary veins, empty the blood from the lungs into the left atrium [1].

As person breathes, the blood in the lung capillaries picks up oxygen. This is why the blood leaving the lung through pulmonary veins have a high oxygen content. In the peripheral circulation, the oxygen is extracted by the tissues, this will decrease the oxygen content of the peripheral veins, the right side of the heart and the pulmonary artery [1].

The resistance is low in the pulmonary circulation owing to the highly distensible vessels. The pulmonary artery has shorter course and thinner walls when we compare it to the aorta, it also contains less smooth muscle and elastin. Pulmonary arterioles also have thin walls, they are not capable of vasoconstriction like systemic arterioles. The pulmonary venules and veins are also very thin and possess little smooth muscle [2].

Pulmonary Circulation in resting healthy individuals, appears to have systolic and diastolic pressure in the pulmonary artery about 25 and 10 mm Hg, respectively, and the mean pressure is about 15 mm Hg. The mean pressure in the left atrium is about 5 mm Hg, and so the total pulmonary arteriovenous pressure gradient is only about 10 mm Hg. The mean hydrostatic pressure in the pulmonary capillaries lies between the pulmonary arterial and pulmonary venous values but somehow it’s closer to the latter [2].

Pulmonary hypertension is considered when the mean pulmonary artery pressure at rest is equal or more than 25 mm Hg [3]. The second WHO conference on pulmonary hypertension, held in Evian, France, in 1998 [4], classified pulmonary hypertension based on similarities in the clinical features [4] and was revised in Venice, Italy, in 2003 to reflect a treatment-based approach to pulmonary hypertension classification [5]. The 4th world symposium on pulmonary hypertension took place in Dana Poi 2008 and provided slight modifications to the classification scheme [6]. This categorization is detailed further in our discussion (Figure 1).