Address for Correspondence :Bibhuti B. Das, Division of Pediatric Cardiology, Kochair Children's Hospital, University of Louisville, Louisville, KY-40202, USA. E-mail: bdas99@hotmail.com

The pediatrician plays an important role in the care of children with PH. Annual influenza vaccination as well as pneumococcal vaccination is recommended unless there are contraindications. Patients should receive adequate antipyretic and antitussive medications during upper respiratory infections. However, decongestants with pseudoephedrine should be avoided as they may exacerbate PH. Diet and/or medical therapy should be used to prevent constipation, since the Valsalva maneuver transiently decreases venous return to the right side of the heart and may precipitate a syncopal episode.

The therapeutic approach to the child with PH begins with a thorough identification of the underlying cause(s) with treatment directed at the underlying cause. These may include use of supplemental oxygen for patients with parenchymal lung disease, early surgical repair of cardiac defects, anti-inflammatory therapy for collagen vascular disease, continuous positive airway pressure therapy and adeno-tonsillectomy for patients with obstructive sleep apnea, and anticoagulation for chronic thromboembolic disease. Nearly all children with PH may need at least some "conventional therapy": oxygen, anticoagulants, digoxin, and diuretics. An overview of current approach and guidelines for treatment is shown in Figure-2.

Figure 2: Current treatment strategies for children with PH

Therapy of PH is targeted against three well-characterized vascular changes: vasoconstriction, thrombus formation, and proliferation of smooth muscle or endothelial cells in the pulmonary vessels. Although, the principles of treatment of PH in children are often derived from observations in adults, data from adults are not easily extrapolated to children. Treatment of children with PH remains individualized based on the underlying etiology, functional class and vasoreactivity of pulmonary vasculature as determined by cardiac catheterization. Commonly used targeted PH therapy for children, their mechanism of action, dose/therapeutic range and side effects are described in table-1.

Table-1: Common targeted therapeutic agents for children with PH

Despite advances in medical management of PH, patients are either non-responsive to such therapies or develop treatment-refractory disease. Patients, who often have marked symptoms of right heart failure or recurrent syncope, have limited treatment options. Atrial septostomy which facilitates right-to-left shunting can sometimes improve cardiac output and may contribute to improved survival for patients with refractory pH. (6) Atrial septostomy may also serve as a bridge to transplantation. Single or bilateral lung transplantation, as well as heart-lung transplantation, has been performed in patients with refractory PH. Results to date are highly variable, and the optimal transplantation procedure or patient selection criteria are yet to be established in children. (7) In practice, patients with medically refractory PH should be referred for evaluation at a lung transplantation center.

Recent advances in the understanding of molecular genetics, cell biology and pathophysiology, and treatment strategies have revolutionized the care of children with pulmonary hypertension, resulting in improved survival and quality of life for these patients. Because of the rarity of this disorder, children with PH merit evaluation and treatment at medical centers with experience in the medical, interventional, and surgical management of this disorder.

Also See Article On "HYPERTENSION (HIGH BLOOD PRESSURE) IN CHILDREN" For More Information

Also See Expertise Views On "Pulmonary Hypertension" Questions

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2. Farber HW, Loscalzo J. Pulmonary arterial hypertension. N Engl J Med. 2004; 351:1655
3. Newman JH, Trembath RC, Morse JA, et al. Genetic basis of pulmonary arterial hypertension: current understanding and future directions. J Am Coll Cardiol. 2004, 43: 33S
4. Simonneau G, Galie N, Rubin LJ, et al. Clinical classification of pulmonary hypertension. J Am Coll Cardiol. 2004, 43, 5S
5. British Cardiac Society Guidelines and Medical Practice Committee. Recommendations on the management of pulmonary hypertension in clinical practice. Heart (British Cardiac Society) 2001, 86 (Suppl 1): II
6. Doyle RL, McCrory D, Channick RN, et al. Surgical treatments/interventions for pulmonary arterial hypertension: ACCP evidence-based clinical practice guidelines. Chest. 2002, 126: 63S
7. Mendeloff EN, Meyers BF, Sundt TM, et al. Lung transplantation for pulmonary vascular disease. Ann Thorac Surg. 2002, 73: 209