In patients with right heart failure (RHF) and pulmonary hypertension (PH), classical teaching often advises cautious diuresis in the setting of ‘preload dependence’ to avoid renal injury and hemodynamic compromise. However, while this physiology may hold true in some clinical settings, such as acute ischemia with right ventricular infarction, it cannot necessarily be extended to PH-related RHF. Rather, in patients with precapillary PH and decompensated RHF, diuresis aimed to decongest the right heart and systemic venous system may be directly beneficial. This study aimed to evaluate the effects of diuresis on renal function and blood pressure in patients with severe precapillary PH. A retrospective chart review was conducted on 62 patients with severe precapillary PH admitted for decompensated RHF. The hemodynamic phenotype of these patients was characterized by invasive hemodynamics and echocardiographic data. Laboratory and hemodynamic data were collected at both admission and discharge. After large-volume diuresis in this patient population, there was an improvement in both glomerular filtration rate and creatinine. While there was a decline in blood pressure after diuresis, this was not clinically significant, given the blood pressure remained in a normal range with improvement in renal function. In conclusion, this study demonstrated that despite concern for preload dependence, significant diuresis in patients with acute decompensated RHF from precapillary PH is not only safe but beneficial.

"Shone's syndrome" was identified in 1963 as a constellation of 4 coexisting lesions leading to left ventricular inflow and outflow obstruction. This congenital heart disease syndrome has hemodynamic consequences throughout the lifetime of patients with this syndrome, and these patients often progress to requiring advanced therapies. This case series reviews the complex decision-making involved in advanced therapy evaluation for this specific patient population. We present 8 cases of patients with Shone's syndrome who were referred for advanced therapies. Of these patients, 6 patients went on to heart transplant alone and 2 patients underwent dual organ transplant. Two patients died after transplant. These cases each highlight important considerations for transplant in this patient population, including the development of pulmonary hypertension, potential need for single vs dual organ transplant, and complex pre- and postoperative courses. We emphasize the importance of early referral for advanced therapies, multidisciplinary evaluation, and individualized care for these complex patients.