hypoplastic left heart syndrome

I would like to get a concensus on the appropriate way to code this diagnosis. Once a patient has had the Norwood, Glenn, and Fontan is it appropriate to code Q234 HLHS as a secondary diagnosis if the patient is admitted for another diagnosis? I feel like it is, the patient is closely monitored for the rest of their life and the left side of their heart is still hypoplastic. My coders do not always agree. I know this was discussed some at conference, just wanted to see how others handle it.


Thanks!

Leah Savage, RN, MSN, CCDS

Kosair Children's Hospital

Louisville, KY

leah.savage@nortonhealthcare.org

Comments

  • If a congenital defect has been repaired, code to the extent any portions or residuals of the defect may remain after surgery.  I  view this as precedence for such complex situations, rendered in ICD-9 era, but concept still applicable.


    Paul Evans, RHIA, CCDS

    VOLUME 21 FIRST QUARTER

    NUMBER 1 2004, Page 16

    Pulmonary Homograft Surgery Status Post Tetralogy of Fallot Repair

    Question: This 8-year-old patient was admitted to our facility with a diagnosis of tetralogy of Fallot, which was repaired six years ago. The patient now presents with right ventricular outflow obstruction, pulmonary insufficiency, significant hypoplasia and takeoff stenosis of the left pulmonary artery. She is referred for operative repair. The patient underwent a right ventricular outflow tract reconstruction with a 25-mm pulmonary homograft; a left pulmonary artery reconstruction out to hilum with enlarging patch arterioplasty; a right pulmonary artery takeoff stenosis arterioplasty; cardiopulmonary bypass and intraoperative transesophageal echocardiogram. What is the proper coding and sequencing of the diagnoses and procedures for this case?

    Answer: Assign code 747.3, Anomalies of pulmonary artery, as the principal diagnosis. Assign codes 746.02, Anomalies of pulmonary valve, stenosis, congenital; V15.1, Other personal history presenting hazards to health, Surgery to heart and great vessels; and V13.69, Personal history of other diseases, Other congenital malformations, as secondary diagnoses. Do not assign a code for tetralogy of Fallot, since this condition was repaired six years ago. Occasionally, after the first complete repair, residual problems may require additional surgery. Assign code 35.25, Replacement of pulmonary valve with tissue graft, for the insertion of the pulmonary homograft in the RV outflow tract; code 39.56, Repair of blood vessel with tissue patch graft, for the left pulmonary artery reconstruction; code 88.72, Diagnostic ultrasound of heart, for the intraoperative echocardiogram; and code 39.61, Extracorporeal circulation auxiliary to open heart surgery, for the cardiopulmonary bypass.

    Coding advice or code assignments contained in this issue effective with discharges April 20, 2004.

    &&

  • I have been told that after all three procedures have been completed than the only correct code to use is Z87.74 Personal history of corrected congenital malformation of heart. A hypoplastic left heart is considered corrected after the three procedures, if there are deficits in cardiac performance left after the procedures, such as residual mitral valve prolapse or other insufficiency than you can code those individually.

    Allie McCullough, RN, MSN, CPN

  • The problem I am having is that the 3 procedures do not fix the hypoplastic left heart, they just allow for the right side of the heart to do all the work. TOF repairs TOF so I agree with coding this as a history of, but the patient with hypoplastic left heart always has a hypoplastic left heart unless they get a transplant. I was hoping for a coding clinic, but there is not one...how can I contact coding clinic?
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  • Thanks! I will :)

    Leah

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