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J Thorac Cardiovasc Surg 2008;136:1095-1097
© 2008 The American Association for Thoracic Surgery

Brief Communication

Mitral annuloplasty in an infant with Barth syndrome and severe mitral insufficiency: First case report and determination of annular diameter

Shu-Chien Huang, MDa, En-Ting Wu, MDb, Shuenn–Nan Chiu, MDb, Wuh-Liang Hwu, MDc, Mei-Hwan Wu, MD, PhDb, Shoei-Shen Wang, MD, PhDa,*

a Department of Surgery, National Taiwan University Hospital, Taipei, Taiwan
b Department of Pediatrics, National Taiwan University Hospital, Taipei, Taiwan
c Department of Genetics, National Taiwan University Hospital, Taipei, Taiwan

Received for publication November 25, 2007; accepted for publication January 17, 2008.

* Address for reprints: Shoei-Shen Wang, MD, PhD, Department of Surgery, National Taiwan University Hospital, 7 Chung-Shan S Rd, Taipei 100, Taiwan. (Email: sswang{at}ha.mc.ntu.edu.tw).

Barth syndrome, an X-linked recessive disorder, is caused by mutations in the taffazin gene (TAZ) at Xq28, leading to severe cardiolipin deficiency in the mitochondrial membrane.1Go The most common presentation is cardiomyopathy in infancy, including left ventricular (LV) dilation, hypertrophy, and noncompaction, with congestive heart failure.

In the case of refractory heart failure, a heart transplant is considered to be required.2Go We performed a successful mitral valve repair on an infant with Barth syndrome with a presentation of cardiogenic shock. We describe the techniques and consideration for mitral valve repair for this infant.

Clinical Summary

An 11-month-old boy had Barth syndrome, which had been diagnosed during his early infancy. Echocardiography revealed LV noncompaction with hypertrophy and impaired systolic function (LV ejection fraction 36%) with severe mitral regurgitation (MR). He was treated with furosemide, captopril, and aspirin. He was also on the waiting list for a heart transplant.

The patient was admitted to the intensive care unit with the impression of cardiogenic shock. Echocardiography showed severe MR with dilated left atrium and LV (Go Figure 1, A). A short period of cardiac massage was required for profound shock and bradycardia; the cardiac surgeon was then immediately consulted regarding placement of a mechanical circulatory support bridge to transplant. We offered mitral valvoplasty as an alternative treatment, because donors for infant heart transplants is extremely rare in our country. Before surgery, the patient's heart rate was 170 beats/min, and his blood pressure was only 60/30 mm Hg, despite an infusion of dopamine (10 µg/[kg · min]) and milrinone (0.5 µg/[kg · min]). After cardiopulmonary bypass, the mitral valve was exposed through the dilated left atrium. We made a partial ring (Go Figure 2) from an expanded polytetrafluoroethylene patch (GORE-TEX cardiovascular patch, 0.4-mm thickness; W.L. Gore & Associates, Inc, Flagstaff, Ariz) for reductive annuloplasty. The inner diameter was 16 mm, and the width was 2 mm. With interrupted 3-0 braided polyester suture (Ethibond; Ethicon, Inc, Somerville, NJ), the expanded polytetrafluoroethylene ring was fixed on the mitral annulus, except in the intertrigone region. The saline solution test confirmed no leakage after the procedure. The patient was smoothly weaned from cardiopulmonary bypass after a bypass time of 76 minutes and an aortic crossclamp time of 33 minutes.


Figure 1
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  		Figure 1. Echocardiography. A, Preoperative echocardiography revealed dilated left atrium (LA), left ventricle (LV), and gap between anterior and posterior mitral leaflets (arrow). B, Three months after surgery, left atrium (LA) and left ventricle (LV) had decreased in size, and minimal mitral valve regurgitation was noted. Single functional anterior mitral leaflet was consistent with our preoperative planning. PML, Posterior mitral leaflet.

 

Figure 2
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  		Figure 2. Schematic drawing of surgical procedure. A, Two concentric circles were drawn on expanded polytetrafluoroethylene cardiovascular patch, with inner diameter of 16 mm and outer diameter of 20 mm. B, Then 2-mm wide expanded polytetrafluoroethylene ring was made. Partial ring was then fixed on mitral annulus with interrupted 3-0 braided polyester suture, thus reducing mitral valve diameter from 25 to 16 mm.

 
After surgery, the patient's blood pressure was 80 to 90 mm Hg, with a left atrial pressure of 8 to 10 mm Hg and a central venous pressure of 10 to 12 mm Hg. He was extubated smoothly 2 days after surgery. Comparing the preoperative and postoperative (3 months after surgery) echocardiographic data, the LV end-diastolic diameter decreased from 4.0 to 3.4 cm (reference 2.6 cm), the left atrial diameter decreased from 3.3 cm to 1.7 cm, and the ejection fraction remained around 35% (Figure 1, B).

Discussion

To our knowledge, this is the first report of mitral valve surgery on an infant with Barth syndrome and severe heart failure. Although we found no data on mitral valve surgery in patients with genetic cardiomyopathy, mitral valve plasty is used as a surgical alternative to heart transplant in patients with ischemic or dilated cardiomyopathy. In this patient, although the LV ejection fraction remained about 35%, the left atrium and LV significantly decreased in size. This finding is consistent with the reverse remodeling of the LV that occurs after amelioration of MR.3Go

Planning of the mitral valve repair in this patient was based on echocardiographic findings. On long-axis views, the regurgitating jet came from noncoaptation of the anterior mitral leaflet (AML) and posterior mitral leaflet, the result of dilation of the annulus and tethering of the posterior mitral leaflet. The annular diameter was measured as 25 mm, and the AML length was 20 mm (Figure 1, A). Because the reduction annuloplasty did not correct the tethering of the posterior mitral leaflet but rather augmented it,4Go we needed to make the AML the sole functioning leaflet with good coaptation height. We therefore decided on a final annular diameter of 16 mm, which would likely result in a zone of coaptation for the valve larger than 4 mm. Postoperative echocardiography confirmed our expectation; the AML was the sole functioning leaflet after the surgery.

A commercially produced pediatric annular ring was unavailable. Although some surgeons use a pericardial strip to make a mitral ring for adults,5Go we used a GORE-TEX patch to form a ring, which we thought would both be a fixed length and preserve some flexibility.

In conclusion, we report our experience with mitral valve repair in an infant with Barth syndrome and severe MR. On the basis of echocardiographic assessment and with surgical planning, reductive mitral annuloplasty was able to be used as an alternative to a heart transplant for this infant with severe MR and heart failure of genetic cardiomyopathy.

References

  1. Spencer CT, Bryant RM, Day J, Gonzalez IL, Colan SD, Thompson WR, et al. Cardiac and clinical phenotype in Barth syndrome. Pediatrics 2006;118:e337-e346.[Abstract/Free Full Text]
  2. Mangat J, Lunnon-Wood T, Rees P, Elliott M, Burch M. Successful cardiac transplantation in Barth syndrome–single-centre experience of four patients. Pediatr Transplant 2007;11:327-331.[Medline]
  3. Westenberg JJ, van der Geest RJ, Lamb HJ, Versteegh MI, Braun J, Doornbos J, et al. MRI to evaluate left atrial and ventricular reverse remodeling after restrictive mitral annuloplasty in dilated cardiomyopathy. Circulation 2005;112(9 Suppl):I437-I442.[Medline]
  4. Kuwahara E, Otsuji Y, Iguro Y, Ueno T, Zhu F, Mizukami N, et al. Mechanism of recurrent/persistent ischemic/functional mitral regurgitation in the chronic phase after surgical annuloplasty: importance of augmented posterior leaflet tethering. Circulation 2006;114(1 Suppl):I529-I534.[Medline]
  5. Calafiore AM, Di Mauro M, Gallina S, Canosa C, Iaco AL. Optimal length of pericardial strip for posterior mitral overreductive annuloplasty. Ann Thorac Surg 2003;75:1982-1984.[Abstract/Free Full Text]




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