Unique treatment method offers hope to child with severe pulmonary hypertension
When Danny Martinez came to Boston from Florida he was very sick. At the time the young boy was suffering from severe pulmonary hypertension, a type of high blood pressure that affects the arteries in the lungs and the right side of the heart. Because Danny’s pulmonary hypertension was so serious doctors in his home state were unsure of how to save him. Along with his parents they arranged it so he could be transferred to Boston Children’s Hospital where he could receive care from the Pulmonary Hypertension Program. As one of the leading pulmonary hypertension programs in the world, Danny’s new team included cardiologists, radiologists and pulmonologists, each with a specific skill for helping children with pulmonary hypertension. After careful examination it became evident that Danny would need a lung transplant to live. However, at the time he was too critically ill to survive until the operation. To keep him alive until the surgery the Pulmonary Hypertension team devised an innovative treatment plan that would act as a bridge to lung transplant. A new approach to treatment To support his lungs, Danny was fitted with a Quadrox membrane oxygenator, a special lung-assist device that adds oxygen to the blood in the same way a healthy pair of lungs would. This device is almost always used as part of an ECMO machine, a special therapy doctors sometimes use to support a critically ill child. But Danny only needed lung assistance, not full ECMO, so his doc...
Primary graft dysfunction (PGD) is a major cause of morbidity and mortality after lung transplantation. Its incidence has decreased with optimization of lung preservation strategies. It remains, however, an important problem, particularly in patients with pulmonary fibrosis and pulmonary hypertension.1 Its development is generally multifactorial and thus referred to as a syndrome. Biologically, activation of the innate immune system and increased endothelial permeability are the main drivers of pulmonary capillary leak after reperfusion.
We present a series of patients with sarcoidosis associated PH and describe correlation between the pre-transplant hemodynamic data from a right heart catheterization (RHC) and vascular morphologic changes in the native explanted lungs.
Central veno-arterial (VA) ECMO provides reliable cardiopulmonary support in selected patients with end-stage lung diseases and severe pulmonary hypertension or cardiac instability. Ambulation dramatically improves patient outcomes. We investigated the outcomes of a new technique of ambulatory central VA ECMO via tunneled cannula insertion and right thoracotomy.
Pre-transplant irreversible pulmonary hypertension (PH) is a contraindication to heart transplantation (HTx) due to the high risk of post-transplant right ventricular (RV) dysfunction. When ventricular assist devices (VAD) are not available, HTx may be considered in selected patients PH.
There is a lack of evidence to guide appropriate donor sizing in recipients with moderate pulmonary hypertension (PH) awaiting transplant. Best practice suggests to oversize hearts for such recipients to prevent post-operative right ventricular failure.
Cardiac allograft vasculopathy (CAV) is a highly prevalent vaso-occlusive disease that is a leading cause of graft failure and mortality after heart transplantation. While the pathogenesis of CAV remains incompletely understood, histologic evidence suggests that macrophages, which constitute an important part of the innate immune response, may play an important role. Prior studies in pulmonary hypertension have shown that macrophage-derived leukotriene B4 (LTB4) induces proliferation and hypertrophy of human pulmonary artery smooth muscle cells.
ISHLT guidelines recommend serial right heart catheterization (RHC) to survey pulmonary hypertension in patients awaiting heart transplant. In LVAD as BTT patients the ideal surveillance frequency is unknown in those without prior pulmonary hypertension or whose pre-LVAD PVR normalized after surgery. Here, we review the utility of our surveillance RHC protocol.
Pulmonary hypertension (PH) is frequent in HT candidates; identifying patients (pts) with urgent need of HT is challenging. Guidelines suggest diastolic transpulmonary gradient (DPG) ≥ 7 mmHg to differentiate combined (CpC) and isolated (IpC) post-capillary PH, but its clinical relevance in this setting is unknown. The aim of our study is to analyze the prognostic impact of PH classification in pts referred for HT, and the interplay of CpC and IpC with markers of ventricular f unction.
We examined our single-center experience using ECLS as bridge to recovery (BTR), non-transplant surgery (BTNTS), or lung transplantation (BTT) in patients with PH.
Although the surgical level of disease in chronic thromboembolic pulmonary hypertension (CTEPH) is routinely described, no study has systematically correlated the preoperative computed tomography pulmonary angiogram (CTPA) with surgical level.