Pharmacological Update and Emerging Treatments of Pulmonary Hypertension.

Pulmonary hypertension (PH) is defined as elevated pressures in the pulmonary artery and is associated with significant morbidity and mortality. The World Health Organization classifies PH into 5 distinct groups based on underlying etiology, pathology, and modality of treatment. Therapeutic approach may be challenging due to the extensive spectrum of causes and underlying mechanisms mediating PH. The 5 groups include pulmonary arterial hypertension (group 1), PH secondary to left heart disease (group 2), PH secondary to chronic lung disease (group 3), chronic thromboembolic pulmonary hypertension (group 4), and PH due to miscellaneous causes (group 5). Although significant progress has been made in the treatment of group 1 PH, there is a continued need to develop new therapies for all types of PH. Additionally, most treatments currently available improve functional capacity and symptoms but without a significant benefit in mortality. In this review, we aim to describe the various etiologies of PH and their established pharmacotherapies, as well as expand on emerging therapeutic options for each group.

Outpatient Continuous Intravenous Inotropy in the Modern Era.

The use of continuous inotropy in patients with advanced heart failure (HF) has been historically controversial due to the prevailing notion that it will increase mortality. In practice, clinicians have continued to revisit this idea as there remains a lack of treatment options for patients in stage D HF. Clinical trials in the past have generally not shown favorable effects of long-term chronic infusions of positive IV inotropic agents on symptoms and exercise tolerance. However, these older studies which indicated poor outcomes with palliative inotropes may not apply to current practice. Modern trials and case series have shown that milrinone and dobutamine may be safely used in patients who are bridging to device therapy or transplant or for palliation. Broad adoption of mortality-reducing modern guideline-directed medical therapy and implantable cardioverter defibrillators may have contributed to the positive results that contemporary trials have seen with inotrope use. For the stage D HF patient, modern use of outpatient inotropy (OI) can alleviate symptom burden and prolong time spent at home. Additionally, more recent studies and case series suggest that OI can be a reasonable alternative to left ventricular assist device placement for both bridging to transplant or as destination therapy. In the appropriate patient, and according to the patient's informed decision and preference, this may be a viable alternative therapeutic option. Contemporary data suggest that OI should be considered in patients who are being evaluated for advanced therapies.

A Case of "Anaphylaxis" to NovoSeven in a Hemophiliac Patient.

Anaphylaxis is a life-threatening emergency that may be confused with other less serious conditions. The onset of true anaphylaxis typically occurs within minutes following exposure to an offending agent, and it can variably include dyspnea/wheezing, hemodynamic compromise, rash, hives/pruritus, swelling, or gastrointestinal symptoms. The absence of an expected association between exposure(s) and classic symptoms should lead to the consideration of alternative diagnoses. Here, we describe the course of a patient with hemophilia B who developed stridor and wheezing after exposure to the recombinant factor VII, NovoSeven, and tranexamic acid (TXA) for the management of hematomas. Due to a reported prior history of anaphylaxis to multiple factor replacements, the patient's initial management included NovoSeven with steroid/antihistamine prophylaxes and close monitoring with epinephrine at the bedside. Despite the administration of prophylaxis, the patient developed significant stridor, was treated with epinephrine and nebulizers and additional steroids, and was transferred to the intensive care unit. There, a pattern of NovoSeven administration followed variably by wheezing and stridor continued for two days until the patient's respiratory condition was predictable and stable. The patient's subsequent clinical course following transfer to the general medical ward was not consistent with anaphylaxis. This case highlights the importance of evaluating for mimickers of anaphylaxis, especially where only select symptoms such as stridor and wheezing are present without other serious signs of anaphylaxis such as hypoxemia, hypotension, or significant tachycardia.

Emerging Modalities for Temporary Mechanical Circulatory Support in Cardiogenic Shock.

Cardiogenic shock (CS) is a life-threatening medical condition that requires prompt recognition and treatment. The use of standardized CS criteria, such as the Society for Cardiovascular Angiography and Interventions criteria, can categorize patients and guide therapeutic strategies. Temporary mechanical circulatory support (MCS) devices have become valuable tools in the treatment of CS, as they can provide cardiovascular support as a bridge to recovery, cardiac surgery, or advanced therapies such as cardiac transplant or durable ventricular assist devices. The use of MCS should be tailored to each individual patient, focused on a stepwise escalation of circulatory support to support both end-organ perfusion and myocardial recovery. As newer MCS devices reduce myocardial oxygen demand without increasing ischemia, the possibility of recovery is optimized. In this review, we discuss the different modalities of MCS focusing on the mechanism of support and the advantages and disadvantages of each device.