DOI: 10.1111/j.1540-8175.2009.01143.

CME

2010, Wiley Periodicals, Inc.

REVIEW ARTICLE

Parachute Mitral Valve in AdultsA Systematic Overview

Fayaz A. Hakim, M.D., Christopher B. Kendall, R.D.C.S., Mohsen Alharthi, M.D., Joel C. Mancina, R.D.C.S., R.V.T., Jamil A. Tajik, M.D., F.A.S.E., F.A.C.C., and Farouk Mookadam, M.Sc., F.R.C.P.C., F.A.C.C.
Department of Internal Medicine, Mayo Clinic, Rochester, Minnesota, USA; and Division of Cardiovascular Diseases, Mayo Clinic, Scottsdale, Arizona, USA

Parachute mitral valve (PMV) is a rare congenital anomaly of the mitral valve apparatus seen in infants and young children. In most instances PMV is associated with other congenital anomalies of the heart, in particular obstructive lesions of the mitral inow (mitral valve ring) and left ventricular outow tract (subaortic stenosis), and coarctation of aorta and is referred to as Shones complex or Shones anomaly. PMV may also occur as an isolated lesion or in association with other congenital cardiac anomalies. Not much is known about PMV in adults as an isolated anomaly or in association with other congenital cardiac anomalies. We reviewed the literature to identify cases of PMV (isolated or associated with other lesions) in adults, to address prevalence, clinical presentation, diagnosis, treatment, and outcome of such patients. (Echocardiography 2010;27:581-586) Key words: parachute, isolated, mitral valve, outcomes, adults True parachute mitral valve (PMV) is characterized by a unifocal attachment of the mitral chordae tendinae resulting in mitral inow obstruction.1,2 This developmental anomaly is most often associated with other obstructive lesions on the left side of heart (supravalvular mitral ring, subaortic stenosis, and coarctation of aorta) and is known as Shones complex or Shones anomaly.3 Shones complex has been almost exclusively reported in infants and children and the outcome is generally poor, due to the presence of multiple hemodynamically signicant lesions requiring several complex surgical interventions with a high mortality.4,5 The outcome of patients with isolated PMV depends upon the severity of the mitral inow obstruction resulting from this cardiac anomaly. Pathophysiology: A normal mitral valve has two leaets (a larger anterior leaet and a smaller posterior leaet), and chordae tendinae diverge to get inserted into two papillary muscles (anterolateral and posteromedial) (Fig. 1A). PMV exists because the chordae tendinae from both mitral valves leaets instead of diverging to insert into two papillary muscles converge on a centrally placed, single papillary muscle (Fig. 1B). This occurs due to disturbed delamination of the anterior and posterior parts of the trabecular ridge (which normally forms anterolateral and posteromedial papillary muscles respectively) between the 5th and 19th week of gestation, thereby forcing these embryonic predecessors of the papillary muscles to condense into a single papillary muscle.2 The chordae tendinae in PMV are often underdeveloped and hence short, thick, and adherent causing decreased mobility of the valve leaets and reducing the size of mitral orice. Furthermore, narrowing of the interchordal spaces results in a smaller secondary mitral orice causing mitral inow obstruction. Most patients present during infancy with mitral stenosis of variable severity.4 Rarely the chordae tendinae may be long and lax precluding complete coaptation of the leaet cusps, which may even prolapse into the left atrium resulting in mitral regurgitation (MR). Uncommonly there may be no functional abnormality of the mitral valve apparatus. PMV usually occurs either as a part of Shones complex3 or in association with other congenital heart diseases including aortic valve stenosis (32%), atrial septal defects (54%), and hypoplastic left heart (19%).6 Isolated PMV is rare accounting for less than 1% of all cases.6 This systematic overview will focus on PMV in adults as an isolated lesion or in
581

No conict of interest exists. Address for correspondence and reprint requests: Farouk Mookadam, M.Sc., F.R.C.P.C., F.A.C.C., Mayo Clinic College of Medicine, 13400 E Shea Blvd, Scottsdale, Arizona 85255, USA. Fax: 480 301 8018; E-mail: mookadam.farouk@mayo.edu

Hakim, et al.

Figure 1. Photographs, Showing normal mitral valve (A) with chordae tendinea attached to two papillary muscles (ALPM = anterolateral papillary muscle and PMPM = posteromedial papillary muscle) and PMV (B) with single papillary muscle receiving chordae tendinae from both mitral valve leaets.

association with other congenital lesions, but exclusive of Shones complex. Methods: We conducted an electronic database search of Medline and PubMed for English language papers from January 1, 1960 to December 31, 2008 using the search terms: isolated, parachute, mitral valve, adults, and Shones complex. Further search terms incorporating associated lesions, congenital, and cardiac anomalies were used. An independent search was conducted by two qualied librarians using similar search terms. Bibliographies of the retrieved articles were scanned to identify further reported cases. Care was taken to avoid duplication of the cases. We reviewed and analyzed the demographic prole, clinical features, diagnostic modalities, treatment and outcome of adult patients with PMV. All adult patients with PMV occurring either as an isolated lesion or in association with other congenital cardiac lesions were included. Results: Nine cases of adult PMV meeting inclusion criteria were identied in the literature over a 49-year period from January 1960 to December 2008. Table I summarizes demographic data, clinical characteristics, and outcome of each patient. Mean age was 44 17 years (range 2265), the majority of the patients (77.77%) were males. Among nine adult patients identied with PMV, ve (55.5%) had an isolated PMV, and the remainder (44.4%) had an associated congenital cardiac lesions (double orice mitral valve with bicuspid aortic valve and coarctation of the aorta in one patient, bicuspid aortic valve with an insignificant coarctation of the aorta in one patient, ventricular septal defect (VSD) with supramitral ring
582

in one patient and double orice, single ventricle, and pulmonary stenosis in one patient. One adult patient with complete Shones complex and one patient with incomplete Shones anomaly were identied during our search. The patient with complete Shones was excluded from the study. Clinical Presentation: Among adult patients with isolated PMV (Patients 1, 2, 3, 7, and 8), one patient (Patient 2)7 presented with sudden death and was found to have subvalvular stenosis of the PMV on autopsy. Three patients (Patient 1,6 Patient 3;8 Patient 7,9 ) presented with progressive dyspnea. Patient 810 was asymptomatic undergoing evaluation for uncontrolled hypertension. Among adult PMV with an associated congenital cardiac lesion (Patients 4, 5, 6 and 9), atrial brillation was the presenting feature in two patients: one had an associated congenital double orice mitral stenosis, bicuspid aortic valve, and status post coarctation repair (Patient 4)11 ; the second (Patient 5)12 with mild obstructive lesions in both mitral and aortic valves (bicuspid aortic valve) and insignicant coarctation of aorta. One patient (Patient 9),13 with double orice, single ventricle, and pulmonary stenosis presented with progressive shortness of breath due to congestive heart failure and one patient with a subaortic VSD (Patient 6),14 presented with an asymptomatic holo-systolic murmur. Diagnosis: Seven (78%) of the nine patients were diagnosed by echocardiography. Six patients had both transthoracic echocardiography (TTE) and transesophageal echocardiography (TEE). An incremental diagnostic value of TEE has been described in two (Patients 3 and 4).8,11 In

TABLE I

Demographic Characteristics, Clinical Features, Diagnostic Modalities, Treatment and Outcome in Patients with PMV in Adults
Diagnosis of PMV Presenting Complaints Dyspnea hemoptysis Sudden death Symptoms of CHF Single papillary muscle Recurrent palpitation snap TEE: PMV, BAV Jaundice Palpitation brillation LA thrombus Asymptomatic murmur, mid-diastolic murmur Worsening SOB s/p PPM, CHB Asymptomatic Hypertension Worsening SOB Palpitation irregular pulse murmur and Apical pansystolic MR, AF and CHF High BP Uncontrolled Home oxygen PPH; s/p AVR, ring TTE, TEE: PMV, Severe MS TTE: PMV, MVP with MR (mild) TTE, TEE: DISV, PMV with severe MR, mild MS, PS Medication for AF and heart failure Echo nding conrmed Hypertension medication NA (mild) Supramitral 3.9); MR VSD, Holo-systolic VSD (Qp/Qs TTE, TEE: PMV, Echo nding conrmed VSD closure, Ring resection, PMV untouched MVR NA Postop 1 yr F/U: Asymptomatic PMV, BAV and rapid atrial MS; TEE: Atrial brillation, Paroxysmal TTE: PMV, mild DC shock Coumadin 2 yrs F/U: Asymptomatic brillation severe MS; with opening atrial orice MV, conrmed Diastolic murmur Paroxysmal TTE: Double Echo nding MVR Uneventful recovery murmur CHF CABG TEE: PMV infarcted Holo-systolic CHF class III s/p TTE: MR (+3); PMV with MS PMV MVR CABG TR, and AR) AR and TR medication Murmurs (MR, Severe MR Mild PMV Heart failure Findings Diagnosis Echo Surgery Autopsy Medical Surgical Clinical Clinical Outcomes Death after 2 yrs Heart failure Sudden death Postop 1 yr F/U: Asymptomatic Treatments

Isolated or

Author

Age/

other

Year

sex

Congenital HD

Glancy et al.6

22/M

Isolated

1971

da Silva and

59/M

Isolated

Edwards7 1973

Shapira et al.8

65/F

Isolated

1995

Yesilbursa et al.11

31/M

CoA (s/p repair) BAV

2000

Double orice MV

Prunier et al.12

33/M

CoA (insignicant)

2001

BAV

Abelson14 2001

28/M

VSD (subaortic)

supravalvular ring

Fitzsimons and

57/F

Isolated

Koch9 2005

Patsouras et al.10

55/M

Isolated

2007

Park et al.13 2007

41/M

DISV PS

Parachute Mitral Valve in Adultsa Systematic Overview

N = case number; TTE = transthoracic echocardiography; TEE = transesophageal echocardiography; HD = heart disease; PMV = parachute mitral valve; MR = mitral regurgitation; MS = mitral stenosis; MVP = mitral valve prolapse; MVR = mitral valve replacement; AR = aortic regurgitation; BAV = bicuspid aortic valve; AVR = aortic valve replacement; VSD = ventricular septal defect; CoA = coarctation of aorta; PPH = primary pulmonary hypertension; PPM = permanent pacemaker; CHB = complete heart block; BP = blood pressure; SOB = shortness of breath; CHF = congestive heart failure; CABG = coronary artery bypass grafting; Postop = post-operative; F/U = follow-up; DISV = double inlet, single ventricle; PS = pulmonary stenosis; NA = not applicable.

583

Hakim, et al.

Patient 3, a preoperative TTE failed to diagnose PMV but intraoperative TEE diagnosed a PMV with a single papillary muscle.8 In Patient 4, TTE showed severe mitral stenosis with a double orice mitral valve, and intraoperative TEE was superior in dening the anatomy of the subvalvular apparatus with a single papillary muscle that supported both mitral valve orices with a parachute feature.11 In Patient 5, a TTE showed characteristic parachute leaets with a shortened chordae tendinea converging into a single papillary muscle in the parasternal long-axis view, and thickened mitral valve leaets and thickened and dysplastic chordae tendinea in the apical fourchamber view. TEE further revealed a bicuspid aortic valve and a left atrial thrombus.12 In Patient 6, both TTE and TEE revealed a supramitral ring and a single papillary muscle in fourchamber views.14 In Patient 7, both TTE and TEE were performed, and TEEs mid-esophageal fourchamber view showed a pear-shaped mitral conguration.9 In Patient 8, only TTE was performed. The parasternal long-axis view showed prolapse of both mitral leaets with mild MR, while the parasternal short-axis view at the mitral valve level showed an eccentric nonstenotic mitral valve orice, and at the papillary muscle level a large posteromedial papillary muscle that received all the chordae was seen. An apical long-axis view showed a typical parachute deformity of the mitral valve with a normal opening and common attachment of all the chordae. The chordae were elongated which was atypicaltypically the chordae are shortened and thickened.11 Two patients (Patients 1 and 2)6,7 had the diagnosis of PMV made at autopsy. Hemodynamic Consequences of the Adult PMV: Four (44%) patients had mitral subvalvular or valvular stenosis: three had either severe (Patients 2 and 7)7,9 or mild (Patient 5)12 subvalvular stenosis, and one patient (Patient 4)11 had a severe mitral valve stenosis with a congenital double orice mitral valve. Five (56%) patients had MR: one (Patient 1)6 had severe mitral valve regurgitation with calcium noted on uoroscopy, one (Patient 9)13 had severe MR associated with mild mitral stenosis, and another (Patient 3)8 with severe mitral valve regurgitation due to infarction of the single papillary muscle. In this patient the PMV was functionally normal until infarction occurred. The remainder had mild MR with (Patient 8)10 or without (Patient 6)14 mitral valve prolapse. Treatments and Prognosis: Mitral valve replacement (MVR) was performed in three (38%) patients: Patient 3 with severe
584

mitral valve regurgitation secondary to ischemic papillary muscle dysfunction underwent MVR combined with coronary artery bypass grafting. At 1-year follow-up she was asymptomatic.8 Patient 4 with signicant mitral stenosis in a double orice underwent MVR with a 31-mm Carbomedics (Austin, TX, USA) prosthesis without complication and no long-term follow up course was mentioned.11 Patient 7 with signicant mitral subvalvular stenosis underwent MVR with a CarpentierEdwards pericardial mitral valve (Edwards Lifesciences, Irvine, CA, USA) and no follow-up course was described.9 Patients without surgical correction of the PMV are described below: One patient (Patient 2) with hemodynamically signicant subvalvular stenosis presented with sudden death.7 Patient 1 presented with dyspnea, combined with severe MR and mild aortic regurgitation he was medically managed for 2 years eventually dying of heart failure.6 Patient 5 presented with rapid atrial brillation, sinus rhythm was restored by an external electric shock and was asymptomatic during the 2 years follow-up.12 Patient 6 presented with a large subaortic VSD undergoing VSD patch closure, and supramitral ring resection at which time the presence of a PMV was conrmedit was left untouched. At 1-year follow-up, the patient remained asymptomatic with a small residual VSD and mild MR.14 In Patient 8, the diagnosis of an isolated PMV was made incidentally during investigations for hypertension.10 One patient (Patient 9) with associated double orice single ventricle and pulmonary stenosis refused surgical interventions and was lost to follow-up.13 Discussion: From the literature search spanning almost ve decades it appears that PMV is rarely seen in the adult population. The majority is male (seven out of nine). Isolated PMV was seen in ve out of nine of this group. PMV in association with other congenital lesions was seen in four out of nine of patients. Combined bicuspid aortic valve and coarctation of the aorta was seen in two patients.11,12 Compared to pediatric PMV,4 concomitant cardiac abnormalities are uncommon in adult PMV. We speculate that this is because combined complex lesions present early in life and usually requires multiple surgical corrections with high mortality. Adults with PMV represent a smaller group of patients with milder lesions who escape detection until adulthood. Furthermore, the condition may be underdiagnosed in asymptomatic adults who never have the need for echocardiography. Finally adults with PMV even after echocardiography may not have a diagnosis conrmed. The diagnostic criteria for PMV are shown in Table II.

Parachute Mitral Valve in Adultsa Systematic Overview

TABLE II Diagnostic Characteristics of PMV Pathology Single papillary muscle Shortened/thickened chordae tendinea converge into a single papillary muscle or one major papillary muscle LV short-axis view At mid-papillary level: single papillary muscle At basal level: Parachute leaets LV long-axis view Shorten /thickened (typical) or elongated chordae converge into a single papillary muscle Four-chamber view Thickened mitral valve leaets and thickened and dysplastic chordae tendinea Pear-shaped mitral conguration with a diastolic dome shape

Echocardiography

Figure 3. Transesophageal echocardiogram (transgastric 0 degree view) of the mid-level of the LV short axis shows a thickened single papillary muscle (arrow) in posteromedial part of the left ventricle (LV). RV = right ventricle; ANT = anterior wall of the LV.

LV = left ventricular.

Adult PMV may have normal hemodynamics (three out of nine) or Doppler evidence of signicant stenosis (three out of nine) or regurgitation (three out of nine). Only one adult patient with complete Shones complex15 was identied during our search suggesting that in general Shones anomaly presents early or are fatal during infancy or childhood. Echocardiography establishes the diagnosis in the majority of the patients with PMV (77.77%). The typical parachute deformity of the mitral valve is best demonstrated in parasternal short axis views of the left ventricle (LV): a single papillary muscle is conrmed at the mid- level of LV (Fig. 2), and the typical parachute leaets are noted at the basal level short axis view. In addition, a long axis of the LV conrms a single papil-

lary muscle accepting all the chordae tendinae insertions. Currently, two-dimensional Doppler echocardiography is the diagnostic method of choice16 whereas TEE is conrmatory in more challenging cases suspected on TTE imaging (Figs. 3 and 4).8,9,12,14 This study shows the incremental value of TEE compared with TTE in two (28.57%) out of seven echo cases.8,11 The PMV may have mimickers such as a pseudo-parachute or parachute-like-mitral valve where chordae tendinae are attached to major papillary muscles and the other being hypoplastic and close to the major one. Careful interrogation by echocardiography that often requires TEE will identify the differences between true parachute and parachute-like mitral valve. Echocardiography helps to dene the functional status of the mitral valve and to dene other associated cardiac anomalies. MRI and multidetector computed tomography may be

Figure 2. Transthroacic echocardiogram of patient with PMV (A) compared with normal mitral valve (B) at the papillary muscle level of LV short axis view. LV = left ventricle; RV = right ventricle; SPM = single papillary muscle; PM = posteromedial papillary muscle; AL = anterolateral papillary muscle.

Figure 4. Transesophageal echocardiogram (transgastric 109 degree view) reveals single papillary muscle, where all the chordae tendinae inserted (arrow). LV = left ventricle; LA = left atrium; ANT = anterior wall of the LV.

585

Hakim, et al.

reasonable complementary imaging techniques in patients with poor echocardiographic acoustic qualities.17 Adult patients with isolated PMV usually present with dyspnea and have hemodynamically signicant lesions of variable severity across mitral valve.6,8,9 However, may be incidentally diagnosed during echocardiography with normal hemodynamics across the mitral valve.10 Such patients generally require no medical or surgical treatment. MVR or repair when feasible needs to be performed only in those patients with hemodynamically signicant stenosis or regurgitation.18 Surgical correction of associated congenital cardiac lesion should be performed only if such lesions are hemodynamically signicant and account for symptoms. Limitations: This study is a systematic overview of literature, which by denition means that asymptomatic cases will likely be under represented. The rarity of adult PMV lends itself nicely to scrutiny with the research methodology of a qualitative systematic overview. Conclusions: Adult PMV is an uncommon condition with only nine cases identied after a systematic literature review over the last half century. Asymptomatic patients may be discovered incidentally. Mitral stenosis is the usual abnormality in symptomatic patients, with atrial brillation or dyspnea being the presenting symptoms. Sudden death can occur. Half of the cases identied required MVR. These ndings are in contrast to the pediatric age group. An international registry of adults with PMV from the American and European societies of echocardiography will go a long way to improve diagnosis and give insights into the natural history of this uncommon condition. References
1. Davachi F, Moller JH, Edwards JE: Diseases of the mitral valve in infancy. An anatomic analysis of 55 cases. Circulation 1971;43:565579.

2. Oosthoek PW, Wenink AC, Wisse LJ, et al: Development of the papillary muscles of the mitral valve: Morphogenetic background of parachute-like asymmetric mitral valves and other mitral valve anomalies. J Thorac Cardiovasc Surg 1998;116:3646. 3. Shone JD, Sellers RD, Anderson RC, et al: The developmental complex of parachute mitral valve, supravalvular ring of left atrium, subaortic stenosis, and coarctation of aorta. Am J Cardiol 1963;11:714725. 4. Schaverien MV, Freedom RM, McCrindle BW: Independent factors associated with outcomes of parachute mitral valve in 84 patients. Circulation 2004;109:2309 2313. 5. Bolling SF, Iannettoni MD, Dick M, 2nd, et al: Shones anomaly: Operative results and late outcome. Ann Thorac Surg 1990;49:887893. 6. Glancy DL, Chang MY, Dorney ER, et al: Parachute mitral valve. Further observations and associated lesions. Am J Cardiol 1971;27:309313. 7. da Silva CL, Edwards JE: Parachute mitral valve in an adult. Arq Bras Cardiol 1973;26:149153. 8. Shapira OM, Connelly GP, Shemin RJ: Ischemic papillary muscle dysfunction in an adult with a parachute mitral valve. J Cardiovasc Surg (Torino) 1995;36:163165. 9. Fitzsimons B, Koch CG: Parachute mitral valve. Anesth Analg 2005;101:16131614. 10. Patsouras D, Korantzopoulos P, Kountouris E, et al: Isolated parachute mitral valve as an incidental nding in an asymptomatic hypertensive adult. Clin Res Cardiol 2007;96:3841. 11. Yesilbursa D, Miller A, Nanda NC, et al: Echocardiographic diagnosis of a stenotic double orice parachute mitral valve with a single papillary muscle. Echocardiography 2000;17:349352. 12. Prunier F, Furber AP, Laporte J, et al: Discovery of a parachute mitral valve complex (Shones anomaly) in an adult. Echocardiography 2001;18:179182. 13. Park SJ, Kwak CH, Hwang JY: Long-term survival in double inlet left ventricle combined with pulmonary stenosis and parachute mitral valve: A rare case. Int Heart J 2007;48:261267. 14. Abelson M: Parachute mitral valve and a large ventricular septal defect in an asymptomatic adult. Cardiovasc J S Afr 2001;12:212214. 15. Koelble N, Weiss BM, Wisser J, et al: Shones anomaly complicated by ascending aortic aneurysm in a pregnant woman. J Cardiothorac Vasc Anesth 2001;15:8487. 16. Grenadier E, Sahn DJ, Valdes-Cruz LM, et al: Twodimensional echo Doppler study of congenital disorders of the mitral valve. Am Heart J 1984;107:319325. 17. Ucar O, Vural M, Cicekcioglu H, et al: Mutidetector CT presentation of parachute-like asymtetric mitral valve. Br J Radiol 2008;81:266268. 18. Coles JG, Williams WG, Watanabe T, et al: Surgical experience with reparative techniques in patients with congenital mitral valvular anomalies. Circulation 1987;76 (3 Pt 2):III117III122.

586