Background
The Ross procedure is a type of valve surgery where the patient’s pulmonary valve is removed and switched to the aortic position. In the pulmonary position , a homograft is placed. The Konno modification of the Ross procedure allows an autograft aortic valve replacement in children with annular and subannular hypoplasia. The Ross-Konno procedure enables potential for growth of the pulmonary autograft in the aortic position. The lifelong anticoagulation can be avoided [1,2].
Meta-analysis of thirty-nine studies (n=5031) shows low early mortality ( 3,0%) and significant reoperation rate after the first postoperative decade due to autograft deterioration (1,15% /patient-year) or right ventricular outflow tract homograft deterioration (0,91%/patient-year) [3].
The endocarditis risk in GUCH patients is 15-140 times higher than in the general population [4]. The Ross procedure seems to be associated with lower incidence of infective endocarditis compared to traditional aortic replacement procedures. Gamez et al. report four cases of infective endocarditis, that occurred in patients with a previously dysfunctioning graft. One patient required surgery, none of patients died during follow-up [5].
Case presentation
A 28-year-old man after correction of congenital aortic stenosis was admitted to our hospital. Previous surgery included: balloon aortic valvuloplasty (7th and 8th year of life) and the Ross-Konno procedure (14th year of life). The patient suffered from fever of unknown origin since two months. Concomitant diseases included advanced dental caries . Current treatment included amoxicillin 500mg t.i.d. The patient reported marked limitation of physical activity (NYHA class III) and weight loss. Physical examination revealed a harsh crescendo-decrescendo ejection murmur, that was the best heard at the left parasternal second intercostal space, BP was 120/80 mmHg, HR 72 bpm, regular normal breath sounds were audible, no peripheral oedema was present. Laboratory tests revealed mild normocytic anemia, strong inflammatory reaction and elevated NT-proBNP level. Resting ECG showed normal heart axis deviation, sinus rhythm with a heart rate of 70 beats/minute and left ventricular hypertrophy.
The transthoracic echocardiography showed enlarged left ventricle size with decreased systolic function. Abnormalities included: presence of small, motile pulmonary vegetation of 5×7 mm, severe regurgitation and moderate stenosis of the pulmonary homograft, moderate regurgitation of the autograft in aortic position. Transesophageal echocardiography confirmed the presence of vegetation.
Methicillin-susceptible Staphylococcus aureus (MSSA) was isolated from blood cultures. In vitro MSSA strains were sensitive to amikacin, gentamicin, tobramycin, ciprofloxacin, levofloxacin, trimethoprim-sulfamethoxazole, and resistant to macrolides, lincosamides and streptogramin B (MLS-B resistance phenotype).The targeted therapy included cloxacillin 12g/day i.v. in 4 doses and gentamicin 3mg/kg/day i.v. in 3 doses.
The patient’s condition improved promptly. He became afebrile after 4 days of therapy.
Two weeks after completion of antibiotic therapy, fever relapsed. The patient complained of cough and dyspnea on exertion. Laboratory tests revealed elevated D-dimer level and strong inflammatory reaction. The transthoracic and transesophageal echocardiography failed to visualise pulmonary vegetation. The chest radiograph showed massive atelectasis in the left lower lobe. Computed tomography scan showed a pulmonary embolus within the posterobasal segments of the left lower lobe. Multiple blood cultures were negative. In treatment of infective endocarditis and septic pulmonary embolism vancomycin 30mg/kg/day i.v. in 2 doses, gentamicin 3mg/kg/day i.v. in 3 doses and ciprofloxacin 800mg/day i.v. in 2 doses were introduced. The patient’s condition improved gradually and no complication was observed up after completion of antibiotic therapy.
Current guidelines
Right-sided infective endocarditis is more frequent in patients with CHD than in acquired heart disease. The distribution of causative organisms does not differ from the pattern found in acquired heart disease. The most common strains are streptococci and staphylococci.
The principal symptoms, complications and basis for diagnosis do not differ from infective endocarditis in general. Transesophageal echocardiography is favored due to complex anatomy and the presence of artificial material.
Treatment of infective endocarditis in CHD follows general principles. Cardiac surgery is appropriate when medical therapy fails, when serious complications arise, and when there is a high risk of devastating septic embolism. Prognosis is better than in other forms of infective endocarditis, with a mortality rate 7 days (e.g. S.aureus, P.aeruginosa) despite adequate antimicrobial therapy (IIa/C)
2. Persistent tricuspid valve vegetations > 20mm after recurrent pulmonary emboli with or without concomitant right heart failure (IIa/C)
3. Right heart failure secondary to severe tricuspid regurgitation with poor response to diuretic therapy (IIa/C)
References
1. Brown JW, Ruzmetov M, Vijay P, et al.The Ross-Konno procedure in children: outcomes, autograft and allograft function, and reoperations. Ann Thorac Surg. 2006 Oct;82(4):1301-6.
2. Reddy VM, Rajasinghe HA, Teitel DF, et al. Aortoventriculoplasty with the pulmonary autograft: the “Ross-Konno” procedure. J Thorac Cardiovasc Sur. 1996 Jan;111(1):158-65; discussion 165-7.
3.Takkenberg JJ, Klieverik LM, Schoof PH, et al. The Ross procedure: a systematic review and meta-analysis.nCirculation. 2009 Jan 20;119(2):222-8.
4.Habib G, Hoen B, Tornos P, et al. Guidelines on the prevention, diagnosis, and treatment of infective endocarditis (new version 2009): Eur Heart J. 2009 Oct;30(19):2369-413
5.Gamez A, Castillo JC, Bonilla JL, et al. Infective endocarditis after the Ross procedure. Int J Cardiol. 2011 Mar 17;147(3):e53-4.
Expert’s comments:(Written authorization required from each expert)
1. Bogusław Kapelak, MD, PhD
There is no indication for surgery, the patient should be closely observed.
2. Prof. Piotr Podolec, MD, PhD
The patient should be pharmacologically treated and observed every months.
3. Jacek Kołcz, MD, PhD
The immunological consultation should be considered.
Expert’s conclusions:
The patient was qualify for pharmacological treatment and father observation.
Authors:
Lidia Tomkiewicz-Pająk MD, PhD1, Leszek Drabik MD1, Maria Olszowska MD, PhD1, Grzegorz Kopeć MD, PhD1
Experts:
Bogusław Kapelak MD, PhD2, Prof. Piotr Podolec MD, PhD1, Jacek Kołcz MD, PhD3.
1Department of Cardiac and Vascular Disease in John Paul II Hospital, Institute of Cardiology, Faculty of Medicine, Jagiellonian University, Krakow, Poland.
2Department of Cardiac, Vessels Surgery and Transplantology, Jagiellonian University College of Medicine, John Paul II Hospital, Krakow, Poland
3Department of Pediatric Cardiac Surgeryy, Polish-American Children’s Hospital, Jagiellonian University, Krakow, Poland
