To the Editor:-Pulmonary arterial hypertension (PAH) occurs in approximately 2% of patients who present for liver transplantation.  Intraoperative management is difficult and associated with mortality rates of up to 80%.  Different from pulmonary hypertension but equally challenging is the hepatopulmonary syndrome (HPS), a triad of hypoxemia, pulmonary vasodilation, and hepatic dysfunction that occurs in approximately 30% of potential transplantation recipients.  Marked hypoxemia that occurs during surgery often complicates the postoperative course and leads to an increase in perioperative death.  Resolution of pulmonary hypertension and hepatopulmonary syndrome has occurred in some patients after liver transplantation. [5,6] However, because cases are limited in any given institution, factors that predict either intraoperative or perioperative survival have not been identified.  Therefore, many institutions deny these high-risk patients liver transplantation. Apart from liver transplantation, there is no other known management.
To learn more about the natural history and outcome of these special patients, a group of interested investigators has established a database to collect information from multiple liver transplantation centers. The principal investigators are Michael J. Krowka, M.D., at the Mayo Clinic, Rochester, Minnesota, and M. Susan Mandell, M.D., Ph.D., at the University of Colorado, Denver.
Members of the steering committee for The Multicenter Hepatopulmonary/Pulmonary Hypertension Database for Liver Transplant Candidates/Recipients include Gary Abrams, M.D., (Hepatology) University of Alabama, Jeffery Crippin, M.D., (Hepatology) Baylor University, Dallas, Marie Csete, M.D., (Anesthesiology) University of California at Irvine, Andre DeWolf, M.D., (Anesthesiology), Northwestern University, Chicago, John Lake, M.D., (Hepatology) University of California at San Francisco, David Plevak, M.D., (Anesthesiology) Mayo Clinic, Rochester, Minnesota, Jeffery Plotkin, M.D., (Anesthesiology) University of Maryland, Victor Scott, M.D., (Anesthesiology) University of Pittsburgh, James K. Stoller, M.D., (Pulmonary) Cleveland Clinic, Terry Therneau, Ph.D., (Biostatistics) Mayo Clinic, Minnesota, and Russell Weisner, M.D., (Hepatology), Mayo Clinic, Minnesota.
The database will collect information regarding the severity, of liver and pulmonary disease, transplantation status, and outcome. The database will address following issues:
1. Identify factors that predict the survival of patients with PAH or HPS during and after liver transplantation surgery.
2. Identify characteristics that predict pulmonary disease resolution.
3. Determine the natural history of patients with PAH or HPS who do not undergo liver transplantation.
4. Identify cost-effective evaluation of patients with PAH and HPS before liver transplantation.
5. Initiate a multicenter therapeutic trial with medications suggested to be effective in pulmonary hypertension of other etiologies.
All patient data are confidential and will be presented periodically at national meetings. We are currently recruiting patients with liver failure that meet the following criteria for identification of pulmonary hypertension and hepatopulmonary syndrome:
1. Pulmonary hypertension
- Mean pulmonary artery pressures greater than 25 mmHg
- Pulmonary vascular resistance greater than 120 dynes [center dot] s [center dot] cm sup -5
- Pulmonary, capillary wedge pressure less than 15 mmHg
2. Hepatopulmonary syndrome
- PaO2less than 70 mmHg, or hemoglobin saturation less than 92% breathing room air and
- Positive enhanced echocardiogram (left atrial opacification greater than 3 beats after right ventricle opacification) or positive sup 99 Technetium lung scan documenting greater than 5% shunt uptake over the brain or kidneys
Survey forms can be obtained from: Michael J. Krowka, M.D., 200 1st Street, SW, Rochester, Minnesota, 55905; telephone: 507 284–2921; E-mail: Krowka.Michael@Mayo.Edu
M. Susan Mandall, M.D., Ph.D.
Michael J. Krowka, M.D.
Department of Anesthesiology; University of Colorado Health Sciences Center; Campus Box B113; 4200 East Ninth Avenue; Denver, Colorado 80262
(Accepted for publication April 10, 1997.)