Primary pulmonary hypertension (PPH) is a rare disease of unknown aetiology which typically results in right heart failure and death within several years of the onset of symptoms. While there is no cure for PPH, several pharmacological and surgical approaches to treatment have been developed over the past decade which have proved useful in a significant proportion of patients. In particular, vasodilator therapy may produce sustained haemodynamic and symptomatic improvement in up to approximately two-thirds of patients; in the remaining patients, vasodilators may either produce no benefit or result in deterioration. The calcium channel blocking agents are the most widely used oral vasodilators; continuous intravenous infusions of epoprostenol (prostacyclin; prostaglandin I2) have been used in some patients who are refractory to oral therapy, particularly as a bridge to transplantation. While combined heart-lung transplantation has been considered the surgical procedure of choice for severe pulmonary hypertension, single lung transplantation has been performed successfully in a small number of patients, and may be the preferred approach in patients with reasonably preserved right heart function.

Primary pulmonary hypertension.

Platelet-rich plasma: evidence to support its use

https://journal.chestnet.org/article/S0012-3692(12)60137-8/pdf

Antithrombotic therapy in neonates and children: Antithrombotic Therapy and Prevention of Thrombosis, 9th ed: American College of Chest Physicians Evidence-Based Clinical Practice Guidelines.

The role of the endothelium in severe sepsis and multiple organ dysfunction syndrome

http://cardiacanaesthesia.in/assets/img/guidelines/Perioperative-Blood-Transfusion-and-Blood-Conservation-in-Cardiac-Surgery.pdf

Perioperative blood transfusion and blood conservation in cardiac surgery: the Society of Thoracic Surgeons and The Society of Cardiovascular Anesthesiologists clinical practice guideline.

Activation of the clotting system during extracorporeal membrane oxygenation in term newborn infants

Inhaled nitric oxide in patients with critical pulmonary perfusion after fontan-type procedures and bidirectional glenn anastomosis

Fulminant meningococcemia defines a life-threatening disease with acute onset, severe septic shock, and progressive hemorrhagic necrosis of the skin. Despite advances in intensive care, the case fatality rate of this disease is still between 30% and 50%.1-5 Disseminated intravascular coagulation (DIC) with deposition of fibrin and histologically demonstrable widespread microvascular thromboses contributes significantly to the pathogenesis.6-9 Impairment of fibrinolysis caused by elevation of plasminogen activator inhibitor 1 (PAI-1), the physiologic inhibitor of tissue plasminogen activator, is part of these clotting abnormalities and has prognostic significance; the extent of elevation of PAI-1 is correlated to the development of shock, renal impairment, and mortality.10,11

Recombinant Tissue Plasminogen Activator Treatment in Two Infants With Fulminant Meningococcemia

OBJECTIVE--To determine the efficacy of fibrinolytic therapy with tissue plasminogen activator (alteplase) in infants and children with arterial thrombosis after cardiac catheterisation. DESIGN--Use of alteplase (Actilyse) in a protocol with prospective data collection. Alteplase was administered to infants and children with arterial thrombosis after cardiac catheterisation. A dose of 0.5 mg/kg/h was given continuously via a peripheral vein for the first hour followed by 0.25 mg/kg/h till clot lysis occurred or treatment had to be stopped because of bleeding complications. SETTING--University hospital, intensive care unit. PATIENTS--17 consecutive infants and children with femoral artery thrombosis after cardiac catheterisation between 1 April 1988 and 31 October 1991. MAIN OUTCOME MEASURE--Reopening of the vessel. RESULTS--Complete clot lysis was achieved in 16 of 17 patients within 4-11 hours after the start of treatment. In one patient only partial lysis occurred. After complete lysis rethrombosis developed in one patient 15 hours after the end of treatment. Bleeding complications were seen in nine patients. These were restricted to the arterial puncture site, except for one who showed mild epistaxis. Three patients had to be treated with packed erythrocytes. CONCLUSIONS--Alteplase was an effective treatment of arterial thrombosis after cardiac catheterisation in infants and children. Further studies are needed to determine whether lower doses will reduce the frequently observed bleeding complications.

https://heart.bmj.com/content/heartjnl/70/4/382.full.pdf

Tissue plasminogen activator (alteplase) treatment for femoral artery thrombosis after cardiac catheterisation in infants and children.

Muntean W, Zenz W, Finding K, Zobel G, Beitzke A. Inhibitor to factor V after exposure to fibrin sealant during cardiac surgery in a two-year-old child. Acta Prediatr 1994;83:84–7. Stockholm. ISSN 0803–5253



A two-year-old infant developed an inhibitor to factor V after cardiac surgery, with application of fibrin sealant containing bovine thrombin. Investigation of this inhibitor by means of inhibition experiments and immunoblot analysis revealed that the inhibitor reacted strongly with bovine, but only weakly with human factor V. Plasmapheresis proved effective in increasing factor V levels. This patient provides further evidence that exposure to topical thrombin preparations may lead to the development of inhibitors in the postoperative period that may cause bleeding complications.

Gerfried Zobel

Papers

Activation of the clotting system during extracorporeal membrane oxygenation in term newborn infants

Inhaled nitric oxide in patients with critical pulmonary perfusion after fontan-type procedures and bidirectional glenn anastomosis

Recombinant Tissue Plasminogen Activator Treatment in Two Infants With Fulminant Meningococcemia

Tissue plasminogen activator (alteplase) treatment for femoral artery thrombosis after cardiac catheterisation in infants and children.

Inhibitor to factor V after exposure to fibrin sealant during cardiac surgery in a two‐year‐old child