Portopulmonary Hypertension: An Updated Review.

Portal hypertension may have major consequences on the pulmonary vasculature due to the complex pathophysiological interactions between the liver and lungs. Portopulmonary hypertension (PoPH), a subset of group 1 pulmonary hypertension (PH), is a serious pulmonary vascular disease secondary to portal hypertension, and is the fourth most common subtype of pulmonary arterial hypertension. It is most commonly observed in cirrhotic patients; however, patients with noncirrhotic portal hypertension can also develop it. On suspicion of PoPH, the initial evaluation is by a transthoracic echocardiogram in which, if elevated pulmonary pressures are shown, patients should undergo right heart catheterization to confirm the diagnosis. The prognosis is extremely poor in untreated patients; therefore, management includes pulmonary arterial hypertension therapies with the aim of improving pulmonary hemodynamics and moving patients to orthotopic liver transplantation (OLT). In this article, we review in detail the epidemiology, pathophysiology, process for diagnosis, and most current treatments including OLT and prognosis in patients with PoPH. In addition, we present a diagnostic algorithm that includes the current criteria to properly select patients with PoPH who are candidates for OLT.

Myocardial fibrosis detected by magnetic resonance imaging, elevated U-CRP and higher mRSS are predictors of cardiovascular complications in systemic sclerosis (SSc) patients.

INTRODUCTION: In previous studies we showed that prevalence of myocardial fibrosis as assessed by late enhancement on cardiac MRI in SSc patients is 45% and is associated to diffuse disease (dcSSc) and lower left ventricle ejection fraction; microvascular damage defined as decreased perfusion on cardiac MRI after adenosine infusion, was also very frequent (79%). Our aim was to identify baseline characteristics associated to the development of cardiovascular outcomes (heart failure, coronary artery disease, arrhythmias, vasculopathy, elevated systolic pulmonary artery pressure and death) in SSc patients with previously documented myocardial fibrosis and microvascular damage. PATIENTS AND METHODS: We included 62 SSc patients who participated in the study of prevalence of myocardial fibrosis (2008-2010) and in our local SSc cohort. We performed baseline clinical evaluation, cardiac MRI, coronary CT angiography, transthoracic echocardiogram, and yearly clinical and cardiovascular evaluation that included Medsger's severity scale items, electrocardiogram, echocardiogram, chest X-ray or HRCT and spirometry; we registered presence and severity of internal organ involvement and cardiovascular outcomes. Ordinal variables were analyzed using Chi square test and Fisher test when appropriate, numeric variables were compared using Student's t-test or Mann Whitney U when appropriate, logistic regression and Cox proportional hazard ratio were used to perform multivariable analysis. RESULTS: We obtained follow-up information from 62 patients (29 dcSSc, 33 lcSSc), mean follow-up was 43.5 months. Multivariable analysis showed that elevated basal ultrasensitive CRP was associated to mortality (p = 0.004, OR: 11.9, 95% CI 2.1-65.7) and recurrent digital tip ischemic ulcers (p = 0.001, OR 26.8, 95% CI 3,9-181.3) on follow-up. Myocardial fibrosis, particularly in the middle segments (p = 0.01, OR: 11.49, 95% CI 1.6-83), and older age (p = 0.02, OR: 1.11, 95% CI 1.01-1.22) were associated to heart failure on follow-up. Higher maximum mRSS was associated to coronary artery disease (p = 0.02, OR: 1.2, 95% CI 1.02-1.38), while insertion point fibrosis (p = 0.001, OR: 12.5 95% CI 2.7-56.6) was associated to recurrent digital tip ischemic ulcers. CONCLUSIONS: This study shows that myocardial fibrosis, elevated ultrasensitive CRP, and higher maximum mRSS are independent predictors of cardiovascular outcomes in SSc patients. Future studies should focus on early preventive and therapeutic strategies for this group of patients.

Assessment of myocardial fibrosis and microvascular damage in systemic sclerosis by magnetic resonance imaging and coronary angiotomography.

OBJECTIVE: Cardiac involvement in SSc is characterized by myocardial fibrosis, arrhythmias and pericarditis. Prevalence studies have shown variable results. The objective of this study was to determine the prevalence of cardiac involvement in SSc patients using the non-invasive, highly sensitive diagnostic methods of cardiac MRI and coronary angiotomography. METHODS: We included 62 SSc patients and excluded those with heart disease prior to the onset of SSc, renal failure, diabetes mellitus, hyperlipidaemia, arterial hypertension, untreated thyroid disease, cor pulmonale, pregnancy or contraindications to performing cardiac MRI. All underwent clinical and laboratory evaluation, ECG, coronary angiotomography and cardiac MRI. RESULTS: The prevalence of myocardial fibrosis was 45% and was higher in dcSSc (59%) than in lcSSc patients (33%; P = 0.04). The mean left ventricular ejection fraction (LVEF) was lower in patients with myocardial fibrosis (56%) than in those without fibrosis (63%; P = 0.0009); myocardial fibrosis on MRI was more frequent in the basal-septal segments of the LV. Seventy-nine per cent of patients had subendocardial perfusion defects and these were associated with higher ultrasensitive serum CRP values. There was no association of myocardial fibrosis or microvascular damage with atherosclerosis. CONCLUSION: The prevalence of myocardial fibrosis on MRI attributable to SSc is 45%, is more frequent and severe in dcSSc patients, is associated with lower LVEF and affects mainly basal LV walls. Microvascular damage in SSc is common and is associated with elevated ultrasensitive CRP levels. Cardiac damage due to SSc is not associated with coronary artery disease.

A 57 year old man with chronic renal failure and cardiac tamponade who developed ischemic hepatitis.

Ischemic hepatitis is an infrequent entity, usually associated with low cardiac out put. We present a case of a 57 year-old man with chronic renal failure and cardiac tamponade who developed elevation of serum alanine transferase level of 5,054 U/L, aspartate transferase level of 8,747 U/L and lactate dehydrogenasa level of 15,220 U/L. The patient developed hepatic encephalopathy and hypoglycemia. Liver Doppler ultrasound was normal. He was seronegative for HBV and HCV, drugs list was scrutinized for the names of known hepatotoxins. Ischemic hepatitis was diagnosed. The hypoglycemia and encephalopathy were solved and the patient was discharged with normal transaminase levels. Ischemic hepatitis is typically preceded by hypotension, hypoxemia, or both. As one would expect, the most common cause of sustained systemic hypotension is cardiovascular disease. Liver biopsy is usually not necessary. The best treatment is support measures and correct the underlying condition.

Cardiac conduction distrurbances in mixed connective tissue disease

Objetivo: Determinar la prevalencia de trastornos de conducción cardiaca en pacientes con enfermedad mixta de tejido conectivo, atendidos en un instituto de la Ciudad de México y su relación con otras manifestaciones de la enfermedad. Método: Ciento trece pacientes admitidos en el Instituto con diagnóstico de enfermedad mixta de tejido conectivo fueron divididos en aquellos con alteraciones de conducción (n=23) y sin estos (n=90). Durante un período de seguimiento de 10.2 ñ 7.8 años, se examinaron, el curso clínico, duración de la enfermedad, tratamiento, tipos de trastornos de conducción y alteraciones sistémicas. Resultados: Observamos un marcado predominio de mujeres en ambos grupos. Las alteraciones de conducción ocurrieron en cerca de 20 por ciento de los pacientes con enfermedad mixta de tejido conectivo y no encontramos diferencias significativas entre los grupos durante el seguimiento. Como era de esperarse, una diferencia significativa entre ambos fue la desviación del aQRS, relacionado a la presencia del bloqueo de fascículo anterior de la rama izquierda del HH, la más frecuente de las alteraciones de conducción observadas. Durante el seguimiento un paciente del grupo A murió, pero ninguno en el grupo B. Conclusión: Las alteraciones de conducción estuvieron presentes en 20 por ciento, en concordancia con lo referido por otros autores en la literatura. Sin embargo, no participaron en la evolución de la enfermedad.