Congenital heart disease and pulmonary hypertension: pharmacology and feasibility of late surgery
Section snippets
Anatomic and physiologic classification of CHD
Anatomic and physiologic classification of CHD is an important part of the evaluation of the patient with CHD and PH. Anatomically, there are 5 features that should be evaluated including defect type, dimension, net directionality of the shunt, associated cardiac or extracardiac anomalies, and repair status (Table 2).7 Large post tricuspid defects, e.g. ventricular septal defects or patent ductus arteriosus, have a higher incidence of developing pulmonary vascular disease than small defects;
Management of the patient with Eisenmenger physiology
In patients who have a large unrepaired systemic to pulmonary communications with associated cyanosis, elevated PVR and absence of a large left to right shunt, surgical intervention can be fatal even if the patient survives the surgery. And not unexpectedly, if a patient has been taken to the operating room to close a shunt and cannot come off bypass, merely taking down the repair will not necessarily prevent a fatal outcome as the patient may still not be able to come off bypass. Similarly,
Statement of Conflict of Interest
Dr. Erika Berman Rosenzweig has received honoraria from Actelion, Gilead and United Therapeutics for attending scientific advisory board meetings and presenting CME lectures in the past, and has received research/grant support from Actelion, Bayer, Gilead, GSK, Eli Lilly, Novartis and United Therapeutics. None of these relationships are relevant to the content of this manuscript.
Dr. Robyn J. Barst has received honoraria for consultancy from: Actelion, Bayer, Eli Lilly. GSK, Gilead, Ikaria,
References (15)
- et al.
Updated clinical classification of pulmonary hypertension
J Am Coll Cardiol
(2009) - et al.
Ambrisentan for pulmonary arterial hypertension due to congenital heart disease
Am J Cardiol
(2011) - et al.
Congenital heart disease associated pulmonary arterial hypertension: preliminary results from a novel registry
Intern Med J
(2011) - et al.
Pulmonary arterial hypertension in paediatric and adult patients with congenital heart disease
Eur Respir Rev
(2009) - McLaughlin VV, Archer SL, Badesch DB, et al. ACCF/AHA 2009 expert consensus document on pulmonary hypertension a report...
- et al.
Second natural history study of congenital heart defects. Results of treatment of patients with ventricular septal defects
Circulation
(1993) Primary pulmonary hypertension. In: Proceedings of the National Heart, Lung, and Blood Institute Pediatric Cardiology Workshop: Pulmonary Hypertension, Ed., Friedman WF
Pediatr Res
(1986)
Cited by (29)
Management of Pulmonary Hypertension in the Pediatric Patient
2022, Cardiology ClinicsCitation Excerpt :Studies typically report using relatively high doses of CCBs, such as amlodipine 20 to 40 mg daily and long-acting nifedipine 120 to 240 mg daily, but the optimal dosing of drugs in pediatric PAH is still unknown. inhaled Nitric Oxide (iNO) causes smooth muscle relaxation of the pulmonary vasculature by activating guanylate cyclase and increasing cGMP.24 iNO may also prevent platelet adhesion to arterial walls via its antiproliferative effect on smooth muscle.
Going Home with a Patent Ductus Arteriosus: Is it Benign?
2022, Journal of PediatricsCongenital Heart Disease-Associated Pulmonary Hypertension
2021, Clinics in Chest MedicineCitation Excerpt :It may be reasonable to treat with targeted PAH therapies for a period of months and serially reevaluate by catheterization to determine operability or partial operability with intentional creation of a small residual defect as a “pop-off.”24 In rare borderline patients, medical therapy may drop the PVR enough to increase the left-to-right shunt to such an extent that there is significantly elevated PBF and the patient develops signs and symptoms of high-output failure.22 As a result, surgical shunt closure should be expeditiously performed to protect the pulmonary vasculature from high-flow shear stress–related damage.
35 - Childhood Pulmonary Arterial Hypertension
2019, Kendig's Disorders of the Respiratory Tract in ChildrenPulmonary Hypertension in Congenital Heart Disease. Beyond Eisenmenger Syndrome.
2015, Cardiology Clinics
Statement of Conflict of Interest: see page 133.