Relationship between arterial blood pressure and plasma norepinephrine levels in a patient with neurogenic hypertension

This study examined the relationship between arterial blood pressure and plasma norepinephrine levels during 12 hours of monitored sleep in a 35-year-old man in whom neurogenic hypertension developed immediately after surgical removal of a large hemangioblastoma at the obex region of the cervical-medullary junction. Plasma norepinephrine levels throughout the 12-hour sampling period correlated (r = 0.81, p less than 0.01) with mean arterial blood pressures, suggesting that sleep-related changes in blood pressure were dependent on associated changes in sympathetic nervous system activity in this patient with neurogenic hypertension.     

Portal and pulmonary hypertension in a patient with MCTD]

A 42-year-old woman with mixed connective tissue disease (MCTD) died due to the rupture of esophageal varices. The autopsy revealed fresh thrombi in the main trunk of the portal vein. Microscopic examination disclosed wide-spread periportal fibrosis and stenosis of peripheral portal veins without remodeling of hepatic lobular architecture, which was compatible to idiopathic portal hypertension (IPH). Anti-phospholipid antibody was negative. Accordingly it is likely that portal vein thrombosis developed secondary to IPH. In the literature 6 (37.5%) of 16 collagen vascular disease patients with IPH died, and three of them were due to rupture of esophageal varices. Therefore IPH should be considered to be one of the most important complications affecting its grave prognosis in patients with collagen vascular disease. The patient also had had pulmonary hypertension (PH) when the diagnosis of portal hypertension was made. In the literature we found 5 collagen vascular disease patients with both PH and IPH like this case. The most outstanding common clinical feature among these 6 patients was Raynaud's phenomenon associated with positive anti-RNP antibody. Moreover 5 of 6 cases including this case simultaneously developed both PH and IPH. The clinical course of these patients suggests there may be a common pathogenetic factor for these two lesions. A possible candidate involved in the pathogenesis of PH and IPH may be endothelin, one of the vasoactive substances, since its receptor is said to be expressed abundantly in pulmonary and portal vasculatures. However, further investigation is necessary to elucidate the mechanism of PH and IPH in collagen vascular diseases.     

Loperamide therapy in a patient with diarrhea and resistant hypertension

A 65-year-old man was admitted to our hospital for resistant essential hypertension. He had a past history of bowel resections due to Crohn’s disease. We assumed that in this patient insufficient drug absorption might be a cause of resistant hypertension and that even increased antihypertensive regimens would fail to decrease blood pressure. We administered loperamide, which may increase drug absorption from the residual intestine. Blood pressure was successfully decreased, with a concomitant increase in blood concentration of antihypertensive drugs. In patients with impaired drug absorption, we should make an effort to restore drug obstruction before considering increased drug regimens.     

Chest pain in a patient with pulmonary arterial hypertension

Chest pain is a frequent symptom in patients with pulmonary arterial hypertension (PAH). Left main coronary artery (LMCA) extrinsic compression from a pulmonary artery (PA) is an increasingly recognized cause of angina or complications, such as acute myocardial infarction, left ventricular dysfunction, arrythmia, and sudden death.We report the case of a 45-year-old patient with pre-capillary pulmonary hypertension (PH), a patent ductus arteriosus corrected surgically during adolescence, and chronic constrictive bronchiolitis. In 2016, the patient began to report oppressive chest pain and worsening fatigue. Computed tomography coronary angiography (CTCA) showed extrinsic LMCA compression by a dilated PA, which was confirmed by invasive coronary angiography and intravascular ultrasound. After stent implantation, the patient reported symptom resolution, and has been asymptomatic ever since.Imaging studies, in particular CTCA, play an important role in the diagnosis of LMCA compression in patients with PAH. The reported case supports the efficacy and safety of stent implantation as a therapeutic option, as already demonstrated in the literature. It shows the complexity of decision making on the operability of systemic-to-pulmonary shunts and reinforces the importance of continuous diagnostic testing.     

Severe pulmonary hypertension in a patient with Whipple's disease

Rapidly progressive heart failure, in part related to severe pulmonary hypertension, developed in a patient with biopsy-proved Whipple's disease. The patient's pulmonary hypertension progressed despite antibiotic therapy and histologic remission of his intestinal disease. The combination of oral nifedipine and low-flow continuous oxygen led to both short- and long-term increases of at least 2 liters per minute in cardiac output and reductions of more than 10 mm Hg in mean pulmonary artery pressure. Accompanying these hemodynamic changes was an improvement of more than 10 percent in right ventricular ejection fraction. The relationship between this patient's pulmonary hypertension and his Whipple's disease is not known.     

Primary pulmonary hypertension in a patient with HIV infection

Several case-reports and small series suggest a causal relationship between human immunodeficiency virus (HIV) infection and pulmonary hypertension. We report on a HIV seropositive man with a high and stable CD4 lymphocyte count (+/- 600/mm3) who developed severe pulmonary hypertension, not attributable to other known causes. This case report underscores the fact that the degree of immunosuppression secondary to the HIV-infection seems to be of little relevance in the pathophysiology of the syndrome. HIV-infected patients with dyspnoea, not related to pulmonary infection, with exercise intolerance, syncope or precordial pain should receive an electrocardiogram and echocardiographic assessment. The exact pathogenetic mechanism of this rapidly progressive disease and whether anti-viral therapy should be promoted is still under investigation.     

Echocardiographic findings in a patient with primary pulmonary hypertension

This is a report of the echocardiographic findings in a 9-year-old white female with primary pulmonary arterial hypertension confirmed by catheterization and later at autopsy. The reported findings of an absent “a” wave, a flat diastolic E to F slope, and a midsystolic closure of the pulmonic valve were observed. In addition, tricuspid valve prolapse was noted. Prolapse of the tricuspid valve may be part of the mechanism of tricuspid insufficiency in a patient with pulmonary hypertension.     

Pulmonary hypertension in a patient with adult-onset stills disease

Pulmonary manifestations of adult-onset Still’s disease (AOSD) include aseptic pneumonitis, pleural effusions, rarely acute respiratory distress syndrome, and restrictive lung disease. Pulmonary arterial hypertension (PAH) occurs with several rheumatologic diseases, however, has only been reported once in AOSD. We describe a 29-year-old woman with a 9-year history of AOSD, who developed PAH without any other obvious cause. Therefore, we conclude that this is likely a result of pulmonary vascular changes related to AOSD. E. Mubashir and M.M. Ahmed contributed equally to this work.     

Pacemaker-masked hypertension in a patient with mitral stenosis.

Severe hypertension was seen in a patient with mitral stenosis when in sinus rhythm. A large fall in systolic blood pressure and in left ventricular end-diastolic pressure was caused by ventricular pacing. Contrary to the usual situation in mitral stenosis this patient paradoxically is a subject who improved haemodynamically with the loss of sinus rhythm and of atrial systole.     

Renovascular hypertension observed in a patient with antiphospholipid-antibody syndrome

The antiphospholipid-antibody syndrome is associated with an increased incidence of arterial and venous thrombosis. Although renal infarction has been observed in these patients, stenotic lesions of the renal artery associated with the antiphospholipid-antibody syndrome have not been reported. A 47-year-old male with a history of hypertension for 7 years developed blurred vision secondary to thrombotic occlusion of the central retinal artery. Laboratory and radiologic examinations revealed renal dysfunction, a positive anticardiolipin antibody, and narrowing of the right renal artery. Successful percutaneous transluminal renal artery angioplasty resulted in normalization of the blood pressure and recovery of renal function.     

Congestive heart failure in a patient with systemic hypertension

A 70-year-old man developed hypertension many years previously and had a bout with severe congestive heart failure 4 to 5 years before his death. Autopsy showed congestion and edema characteristic of heart failure, an enlarged heart, and slight focal interstitial left ventricular fibrosis, but only slight to moderate coronary atherosclerosis.