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  • 1 University of Foggia, Italy
  • | 2 GVM Care & Research, Italy
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Parachute mitral valve (PMV) is a congenital cardiac malformation where typically all tendons chordae converge and insert into one major papillary muscle. We report the case of 27-year-old man affected by Hodgkin’s lymphoma-mixed cellularity subtype with polychemotherapy treatment and Barrett’s esophagus who came to our attention for routine echocardiographic exam and evaluation of left ventricular function. In the presence of normal left ventricular systolic function and mild-moderate mitral regurgitation, a careful examination of mitral apparatus showed a single papillary muscle suspected for PMV. In the presence of Barrett’s esophagus, the patient was further assessed cardiac magnetic resonance (CMR) that confirmed the diagnosis of PMV and excluded other associated congenital anomalies. Despite the rare incidence of PMV and a diagnosis generally based on transesophageal echocardiography, CMR could represent a useful tool, in selected cases, to confirm the diagnosis of PMV, identify other possible congenital anomalies, and assess ventricular and valvular function.

Abstract

Parachute mitral valve (PMV) is a congenital cardiac malformation where typically all tendons chordae converge and insert into one major papillary muscle. We report the case of 27-year-old man affected by Hodgkin’s lymphoma-mixed cellularity subtype with polychemotherapy treatment and Barrett’s esophagus who came to our attention for routine echocardiographic exam and evaluation of left ventricular function. In the presence of normal left ventricular systolic function and mild-moderate mitral regurgitation, a careful examination of mitral apparatus showed a single papillary muscle suspected for PMV. In the presence of Barrett’s esophagus, the patient was further assessed cardiac magnetic resonance (CMR) that confirmed the diagnosis of PMV and excluded other associated congenital anomalies. Despite the rare incidence of PMV and a diagnosis generally based on transesophageal echocardiography, CMR could represent a useful tool, in selected cases, to confirm the diagnosis of PMV, identify other possible congenital anomalies, and assess ventricular and valvular function.

Background

Parachute mitral valve (PMV) is a congenital cardiac malformation where typically all chordae tendineae converge and insert into one major papillary muscle. This malformation also grouped the cases of two papillary muscles that are too close creating the same hemodynamic effect of single muscle [12] or where there is a dominant papillary muscle with its tip reaching to the valve leaflets generally named as parachute-like asymmetric mitral valve [3]. All these conditions restrict the motion of leaflets and obstruct the blood flow into the left ventricle during diastole causing mitral stenosis or, less commonly, mitral regurgitation. Usually, the anomaly is detected in infants and young children; adult presentation is extremely rare and is generally mild in terms of mitral stenosis [4].

Case Report

We report the case of 27-year-old man affected by Hodgkin’s lymphoma-mixed cellularity subtype with polychemotherapy treatment and Barrett’s esophagus who came to our attention for routine echocardiographic exam and evaluation of left ventricular function. The patient did not report any dyspnea. In the presence of normal left ventricular systolic function and mild-moderate mitral regurgitation (Fig. 1), a careful examination of mitral apparatus showed a single papillary muscle, unusually located between anterolateral and posteromedial papillary muscles normal position, larger than normal: the aspect was extremely suspect for PMV (Fig. 1b). Mild mitral valve prolapse was also present. Indexed left ventricular end-diastolic volume was 68 ml/m2, left ventricular ejection fraction 60%, and left atrium diameter 41 mm (anteroposterior diameter). For further assessment, the patient was referred for cardiac magnetic resonance (CMR) because of the presence of Barrett’s esophagus, which confirmed the diagnosis of PMV and excluded other associated congenital anomalies (Fig. 1c). No sign of aortic coarctation was found.

Fig. 1.
Fig. 1.

(a) Mild-moderate mitral regurgitation with asymmetric jet. (b) Single papillary muscle located between anterolateral and posteromedial papillary muscles usual position. (c) Cardiac magnetic resonance confirming the presence of a single papillary muscle

Citation: Interventional Medicine and Applied Science Interventional Medicine and Applied Science 11, 1; 10.1556/1646.11.2019.03

Discussion

PMV is a rare cardiac congenital malformation that is typically diagnosed in childhood and not unusually associated with other congenital malformations and obstruction of left chambers; this condition, known as Shone’s syndrome, includes supravalvular ring of the left atrium, subaortic stenosis, and coarctation of the aorta. PMV could be associated with other congenital malformations with different rates: aortic coarctation (68%), atrial septal defect (54%), ventricular septal defect (46%), aortic valve stenosis (32%), subaortic stenosis (20%), and left ventricular hypoplasia (19%) [5]. The finding of PMV in adults is rare and often following the clinical diagnosis to mitral stenosis or regurgitation; sometimes, the diagnosis could be an occasional finding.

Rim et al. showed that PMV may severely wrinkle leaflets narrowing interchordal spaces at peak systole, compared to those of the normal MV. Furthermore, there is a complex leaflet coaptation with flatter leaflets deformation and a smaller orifice-to-annulus ratio [6]. All these abnormalities are the cause of mitral stenosis and/or regurgitation in patients with PMV.

In our clinical case, the presence of mitral regurgitation without stenosis is probably the expression of the particular position of papillary muscle that was located closer to ventricular apex than normal; therefore, more elongated tendinous chordae permit a greater excursion of mitral leaflets without valve stenosis.

The diagnosis of this rare congenital malformation is generally suspected with transthoracic echocardiography and confirmed by transesophageal echocardiogram. In our case, CMR was extremely useful for the confirmation of PMV, given the presence of Barrett’s esophagus that usually contraindicates invasive transesophageal examinations. Nevertheless, even in the absence of any contraindication to invasive examinations, CMR may add further information on ventricular dimension, systolic function, degree of mitral regurgitation, and other possible associated congenital abnormalities.

Conclusion

Despite the rare incidence of PMV and a diagnosis generally based on transesophageal echocardiography, CMR could represent a useful tool, in selected cases, to confirm the diagnosis of PMV, identify other possible congenital anomalies, and assess ventricular and valvular function.

Authors’ contribution

All authors have read and approved the final version of the manuscript.

Conflict of interest

The authors have no potential conflict of interest to disclose.

Acknowledgements

This work was performed at the University of Foggia, Department of Cardiology, Foggia, Italy.

References

  • 1.

    Shone JD , Sellers RD , Anderson RC , Adams P , Lillehei CW , Edwards JE : The developmental complex of “parachute mitral valve,” supravalvular ring of left atrium, subaortic stenosis, and coarctation of aorta. Am J Cardiol 11, 714725 (1963)

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  • 2.

    Rosenquist GC : Congenital mitral valve disease associated with coarctation of the aorta: A spectrum that includes parachute deformity of the mitral valve. Circulation 49, 985993 (1974)

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    • Search Google Scholar
    • Export Citation
  • 3.

    Oosthoek PW , Wenink AC , Macedo AJ , Gittenberger-de Groot AC : The parachute-like asymmetric mitral valve and its two papillary muscles. J Thorac Cardiovasc Surg 114, 915 (1997)

    • Crossref
    • Search Google Scholar
    • Export Citation
  • 4.

    López-Pardo F , Urbano-Moral JA , González-Calle A , Laviana-Martinez F , Esteve-Ruiz I , Lagos-Degrande O , López-Haldon JE : Three-dimensional transesophageal echocardiography in the anatomical assessment of isolated parachute mitral valve in an adult patient. Echocardiography 32, 17321735 (2015)

    • Crossref
    • Search Google Scholar
    • Export Citation
  • 5.

    Fitzsimons B , Koch CG : Parachute mitral valve. Anesth Analg 101, 16131614 (2005)

  • 6.

    Rim Y , McPherson DD , Kim H : Effect of congenital anomalies of the papillary muscles on mitral valve function. J Med Biol Eng 35, 104112 (2015)

    • Crossref
    • Search Google Scholar
    • Export Citation
  • 1.

    Shone JD , Sellers RD , Anderson RC , Adams P , Lillehei CW , Edwards JE : The developmental complex of “parachute mitral valve,” supravalvular ring of left atrium, subaortic stenosis, and coarctation of aorta. Am J Cardiol 11, 714725 (1963)

    • Crossref
    • Search Google Scholar
    • Export Citation
  • 2.

    Rosenquist GC : Congenital mitral valve disease associated with coarctation of the aorta: A spectrum that includes parachute deformity of the mitral valve. Circulation 49, 985993 (1974)

    • Crossref
    • Search Google Scholar
    • Export Citation
  • 3.

    Oosthoek PW , Wenink AC , Macedo AJ , Gittenberger-de Groot AC : The parachute-like asymmetric mitral valve and its two papillary muscles. J Thorac Cardiovasc Surg 114, 915 (1997)

    • Crossref
    • Search Google Scholar
    • Export Citation
  • 4.

    López-Pardo F , Urbano-Moral JA , González-Calle A , Laviana-Martinez F , Esteve-Ruiz I , Lagos-Degrande O , López-Haldon JE : Three-dimensional transesophageal echocardiography in the anatomical assessment of isolated parachute mitral valve in an adult patient. Echocardiography 32, 17321735 (2015)

    • Crossref
    • Search Google Scholar
    • Export Citation
  • 5.

    Fitzsimons B , Koch CG : Parachute mitral valve. Anesth Analg 101, 16131614 (2005)

  • 6.

    Rim Y , McPherson DD , Kim H : Effect of congenital anomalies of the papillary muscles on mitral valve function. J Med Biol Eng 35, 104112 (2015)

    • Crossref
    • Search Google Scholar
    • Export Citation

2019  
Scimago
H-index
11
Scimago
Journal Rank
0,220
Scimago
Quartile Score
Medicine (miscellaneous) Q3
Scopus
Cite Score
155/133=1,2
Scopus
Cite Score Rank
General Medicine 199/529 (Q2)
Scopus
SNIP
0,343
Scopus
Cites
206
Scopus
Documents
23

 

Interventional Medicine and Applied Science
Language English
Size  
Year of
Foundation
2009
Publication
Programme
changed title
Volumes
per Year
 
Issues
per Year
 
Founder Akadémiai Kiadó
Founder's
Address
H-1117 Budapest, Hungary 1516 Budapest, PO Box 245.
Publisher Akadémiai Kiadó
Publisher's
Address
H-1117 Budapest, Hungary 1516 Budapest, PO Box 245.
Responsible
Publisher
Chief Executive Officer, Akadémiai Kiadó
ISSN 2061-1617 (Print)
ISSN 2061-5094 (Online)

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