Drug Therapy and Surgery in Congenital Heart Disease With Pulmonary Hypertension

NCT01548950 | Completed DMC

• 1 other identifier: CAPPesq 0502/11
• Study Type: interventional
• Target: 50
• Locations: 1 country
• Sites: 1

Brief Summary

The purpose of this study is to test the hypothesis that treating PAH-CHD patients preoperatively with PAH drugs and keeping them on treatment for six months after surgery reduces the risk of immediate postoperative death and the risk of residual PAH at six months following operation to \<10%.

Conditions

Congenital Heart Disease; Pulmonary Arterial Hypertension

Keywords

Congenital heart disease; Pulmonary arterial hypertension; Sildenafil; Bosentan; Pediatric cardiac surgery

Outcome Measures

Primary Outcomes (1)

• Immediate postoperative right cardiac failure and mortality, and prevalence of residual PAH six months after surgery.

  Drug treatment must reduce the prevalence of immediate postoperative right cardiac failure / death to \<10%, and the prevalence of residual PAH six months after cardiac surgery to \<10%. Residual PAH is defined as an elevation of mean pulmonary artery pressure above 25 mmHg, and elevation of pulmonary vascular resistance above 3.0 Wood units x squared meters (body surface area).

  Six months following surgery

Secondary Outcomes (1)

• Pulmonary vascular resistance six months after surgical repair of congenital cardiac shunts with PAH

  Six months following surgery

Study Arms (1)

Single-arm study

OTHER

Preoperatively, sildenafil until development of pulmonary congestion (1-4 weeks). On treatment pulmonary congestion (dyspnea and need for increasing diuretics) occurs when there is a substantial decrease in pulmonary vascular resistance, which may be confirmed noninvasively by Doppler-echocardiography. At that moment, patient will be assigned to surgery. If pulmonary congestion is not observed, bosentan will be added on top of sildenafil, and the patient will be kept on treatment for 10-12 months. In this case, a new cardiac catheterization will be performed before surgery. In both cases (short-term and medium-term treatment) patients will be kept on treatment for six months following surgery, and then re-catheterized.

Drug: Sildenafil singly or in association with Bosentan

Interventions

Sildenafil singly or in association with Bosentan DRUG

Sildenafil, 1-4 mg/Kg/day (6-hour intervals) preoperatively, until development of pulmonary congestion (generally 1-4 weeks) or preoperatively, for 10-12 months, in association with bosentan (15.6-62.5 mg b.i.d.) if pulmonary congestion does not develop. Surgery will be performed at 1-4 weeks (short-term treatment) or at 10-12 months (medium-term treatment) if operability criteria are met (catheterization). In both cases (short and medium-term treatments), the drug or drugs will be kept for 6 months postoperatively, when final catheterization will be performed for efficacy testing.

Single-arm study

Eligibility Criteria

• Age: 2 Months+
• Sex: all
• Healthy Volunteers: No
• Age Groups: Child (0-17), Adult (18-64), Older Adult (65+)

You may qualify if:

• Potentially operable patients with congenital cardiac septal defects (bi-ventricular physiology) and PAH, must have at least three of the following severity criteria: age \> 18 months; absence of congestive heart failure (pulmonary congestion); Down syndrome; bidirectional shunting across the septal defect; periods of systemic (peripheral) oxygen saturation \< 90%.

You may not qualify if:

• Patients with complex cardiac anomalies for whom there are no possibilities of complete repair. Patients with uni-ventricular physiology.

Sponsors & Collaborators

• University of Sao Paulo General Hospital: lead
• Instituto do Coracao: collaborator
• Fundação de Amparo à Pesquisa do Estado de São Paulo: collaborator

Study Sites (1)

Instituto do Coração (InCor) HCFMUSP

São Paulo, 05403-900, Brazil

Location

MeSH Terms

Conditions

Heart Defects, Congenital; Pulmonary Arterial Hypertension

Interventions

Bosentan

Condition Hierarchy (Ancestors)

Cardiovascular Abnormalities; Cardiovascular Diseases; Heart Diseases; Congenital Abnormalities; Congenital, Hereditary, and Neonatal Diseases and Abnormalities; Hypertension, Pulmonary; Lung Diseases; Respiratory Tract Diseases

Intervention Hierarchy (Ancestors)

Benzenesulfonamides; Sulfonamides; Amides; Organic Chemicals; Benzene Derivatives; Hydrocarbons, Aromatic; Hydrocarbons, Cyclic; Hydrocarbons; Sulfones; Sulfur Compounds; Pyrimidines; Heterocyclic Compounds, 1-Ring; Heterocyclic Compounds

Study Officials

• Antonio Augusto Lopes, M.D.

  Instituto do Coração (InCor) - HCFMUSP - São Paulo - Brazil

  PRINCIPAL INVESTIGATOR

Study Design

• Study Type: interventional
• Phase: not applicable
• Allocation: NA
• Masking: NONE
• Purpose: TREATMENT
• Intervention Model: SINGLE GROUP
• Sponsor Type: OTHER
• Responsible Party: SPONSOR

Study Record Dates

• First Submitted: March 5, 2012
• First Posted: March 8, 2012
• Study Start: September 1, 2011
• Primary Completion: January 1, 2014
• Study Completion: March 1, 2020
• Last Updated: March 30, 2020

Record last verified: 2015-06

Locations

BR (1)