European Journal of Echocardiography 2007 8(1):59-62; doi:10.1016/j.euje.2005.12.003
Copyright © 2005, The European Society of Cardiology
Isolated cleft of the anterior mitral valve leaflet
Ana Timóteoa,*,
Ana Galrinhoa,
António Fiarresgaa,
Luisa Brancoa,
Nuno Banazolb,
Ana Leala,
José Fragatab and
Jorge Quininhaa
aEchocardiography Laboratory, Cardiology Department, Santa Marta Hospital, R. Santa Marta, 1169-024 Lisbon, Portugal
bCardio-thoracic surgery Department, Santa Marta Hospital, Lisbon, Portugal
Received 17 November 2005; received in revised form 25 November 2005; accepted after revision 4 December 2005.
* Corresponding author. Av. Miguel Torga, 27, Edificio C, 9 ° A, 1070-183 Lisbon, Portugal. Tel.: +351 21 359 40 00. ana_timoteo{at}yahoo.com
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Abstract
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Isolated anterior mitral leaflet cleft (not associated with
atrio-ventricular septal defect) is a rare cause of congenital
mitral regurgitation, and the treatment consists of direct suturing
of the cleft. We present a clinical case with this entity.
Keywords: Isolated cleft of the mitral valve; Congenital mitral regurgitation
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Introduction
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Isolated mitral cleft is a rare congenital cause of mitral insufficiency.
1 Its association with other cardiac malformations has been previously
described.
2 The clinical manifestations are those of mitral
insufficiency. Pre-operative diagnosis is sometimes difficult.
Echocardiography shows the cleft and any associated anatomic
anomalies.
1
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Clinical case
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A 32-year old male, with a previous history of surgery to close
a patent arterial duct at the age of 9 years was considered
in subsequent echocardiograms, to have some degree of mild mitral
valve insufficiency due to a probable mitral valve prolapse.
He remained stable over the years, and was completely asymptomatic.
In 2002, became symptomatic with development of slight dyspnoea
and fatigue. The patient improved after starting diuretics.
In 2004, there was a new worsening of his clinical condition
(NYHA class III). The echocardiogram showed the presence of
severe mitral regurgitation and a cleft of the anterior mitral
valve leaflet was evident (
Fig. 1). There was also some
dilatation of the left ventricle (
Table 1). Transesophageal
echocardiography confirmed those findings (
Figs. 2 and 3
). The
degree of regurgitation was quantified as severe, with systolic
inversion of the pulmonary venous flow. There were no other
anomalies.
The patient underwent surgery in January 2005, with confirmation
of the cleft in the anterior leaflet (
Fig. 4). A suture
of the entire length of the cleft was performed with a simple,
interrupted suture. The intra-operative transesophageal echocardiogram
showed very mild mitral insufficiency. At the end of surgery,
the patient had a very severe allergic reaction to protamin,
with cardiac arrest. The remaining post-operative course was
uneventful and he was discharged 15 days after surgery. The
echocardiogram performed on discharge showed a left ventricle
yet dilated, with moderate systolic dysfunction. There was moderate
mitral insufficiency (one central jet and another eccentric,
originating in the middle portion of the anterior mitral leaflet).
We considered an eventual rupture of the suture after surgery,
related with the resuscitation performed. Nevertheless, the
patient is presently in NYHA class I (12 months after surgery),
and is professionally active with no limitations. The 6-month
echocardiogram showed recovery of left ventricular dimensions
and function, but maintenance of the two regurgitant jets described
at discharge.
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Discussion
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Mitral valve clefts not associated with a septal defect of the
endocardial cushion defect type (atrio-ventricular septal defect),
also called isolated mitral cleft, is a rare cause of congenital
mitral insufficiency,
1 described for the first time in 1954.
3 It is defined by the Congenital Heart Surgery Nomenclature and
Database Project as a "cleft of the anterior mitral valve leaflet
not associated with a primum atrial septal defect or other features
of atrio-ventricular septal defect (with or without other associated
defects)".
4 An association with other cardiac anomalies such
as secundum type atrial septal defect, transposition of the
great arteries, ventricular septal defect, tricuspid atresia,
patent arterial duct, coarctation of the aorta, double outlet
right ventricular and anomalous pulmonary venous connection
have been previously described.
2 In the present patient, there
was an association with patent arterial duct.
The usual manifestations are the ones that are found in patients with mitral insufficiency. Pre-operative diagnosis is sometimes difficult, due to the position, dimensions and shape of the cleft. Echocardiography can demonstrate the cleft and the anatomical malformations eventually associated, such as accessory papillary muscles, papillary muscle displacement, laxity/rupture of chordae and thickening of the edges of the cleft with increasing age. At younger ages, clefts cause mild mitral insufficiency. Perier and Clausnizer3 studied older patients and detected more severe degrees of mitral insufficiency, suggesting some worsening with age, as happened in our patient. In our case, the cleft was not identified in early childhood, and the mitral insufficiency worsened in subsequent years, with need for late cardiac surgery. The technique used was the one described for these cases. Unfortunately, an anaphylactic reaction occurred, with need for cardiac resuscitation measures, which could have compromised in part the success of the intervention, eventually by suture rupture. Nevertheless, 6 months after surgery, the patient resumed his professional activity, is in NYHA class I, and has normalized the left ventricular dimensions, in spite of persistence of residual moderate mitral insufficiency (less significant than at the time of surgery).
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References
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- Di Segni E., Kaplinsky E., Klein H. Color Doppler echocardiography of isolated cleft mitral valve. Roles of the cleft and the accessory chordae. Chest (1992) 101:12–15.[CrossRef][ISI][Medline]
- Oshima K., Takahashi T., Sato Y., et al. Mitral regurgitation with an isolated anterior mitral leaflet cleft – a case report. Circ J (2005) 69:114–115.[CrossRef][ISI][Medline]
- Perier P., Clausnizer B. Isolated cleft mitral valve: valve reconstruction techniques. Ann Thorac Surg (1995) 59:56–59.[Abstract/Free Full Text]
- Sulafa A.K.M., Tamimi O., Najm H.K., Godman M.J. Echocardiographic differentiation of atrioventricular septal defects from inlet ventricular septal defects and mitral valve clefts. Am J Cardiol (2005) 95:607–610.[CrossRef][ISI][Medline]
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