Pulmonary arterial and pulmonary venous hypertension develop from distinctly different etiologies. Pulmonary arterial hypertension (PAH), or Group 1 pulmonary hypertension (PH), is a precapillary PH that arises idiopathically or as the result of a divergent array of causes, including connective tissue disease. Pulmonary venous hypertension (PVH), or Group 2 PH, primarily manifests as a postcapillary PH in the setting of left heart failure or valvular disease. A subset of PVH patients, however, develop a reactive precapillary component of PH that mimics PAH. These patients can be misdiagnosed as having Group 1 PH by 2-dimensional echocardiography and are sometimes treated as such, which leads to exacerbation of heart failure. Therefore, 2-dimensional or Doppler echocardiography alone cannot be used to differentiate between these two classifications of PH. This highlights the need for right heart catheterization in the clinical assessment and diagnostic work-up of PH. The combination of imaging and invasive hemodynamic assessment by right heart catheterization provides the best diagnostic approach to ensure proper delineation of pulmonary arterial and pulmonary venous hypertension, and in turn leads to appropriate treatment.
Pulmonary hypertension, which may lead to right ventricular (RV) failure, increases with left ventricular (LV) diastolic dysfunction severity. The prevalence and determinants of RV failure were analyzed in 120 patients admitted with acute left heart (LH) failure. Patients were divided into RV failure (n=50) and non-RV failure (n=70) groups. The prevalence of RV failure was found to be 42%. In both groups, two thirds of the patients had isolated LV diastolic dysfunction and the rest had combined LV systolic and diastolic dysfunction. Patients in the RV failure group were characterized by higher LV diastolic grade (2.2 ± 0.6 vs 1.84 ± 0.7; P=.0070), pulmonary artery systolic pressure (PASP; 57.8 ± 15.3 vs 50.14 ± 12.1 mm Hg; P=.0028), right atrial enlargement (92% vs 25.7%; P=.000001), and more-than-moderate tricuspid regurgitation (58% vs 27.1%; P=.0006). RV failure is a frequent finding in patients with advanced LH failure. It is strongly associated with the severity of LV diastolic dysfunction and the severity of PASP.
We report the case of a 79-y-old male with Tetralogy of Fallot (TOF) who underwent Brock's procedure in his twenties and has survived event-free. Brock's procedure, which entailed infundibular resection and pulmonary valvotomy, has been associated with complications including the need for re-operation, pulmonary regurgitation, and biventricular failure.
Cardiac allograft vasculopathy is one of the leading causes of death following the first 5 years after orthotopic heart transplantation along with late graft failure, likely secondary to undiagnosed CAV. Currently there is no single medical treatment available for this condition except modification of risk factors and immunosuppression. Retrasplantation remains the hope for this entity with some limitations.
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