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J Thorac Cardiovasc Surg 2009;137:385-393
© 2009 The American Association for Thoracic Surgery

Congenital Heart Disease

Parachute mitral valve: Morphologic descriptors, associated lesions, and outcomes after biventricular repair

^a Division of Cardiology in the Departments of Pediatrics, Anesthesiology & Critical Care, and Surgery in the Cardiac Center at The Children's Hospital of Philadelphia, University of Pennsylvania School of Medicine, Philadelphia, Pa
^b Division of Critical Care Medicine in the Departments of Pediatrics, Anesthesiology & Critical Care, and Surgery in the Cardiac Center at The Children's Hospital of Philadelphia, University of Pennsylvania School of Medicine, Philadelphia, Pa
^c Division of Biostatistics in the Departments of Pediatrics, Anesthesiology & Critical Care, and Surgery in the Cardiac Center at The Children's Hospital of Philadelphia, University of Pennsylvania School of Medicine, Philadelphia, Pa
^d Division of Cardiothoracic Surgery in the Departments of Pediatrics, Anesthesiology & Critical Care, and Surgery in the Cardiac Center at The Children's Hospital of Philadelphia, University of Pennsylvania School of Medicine, Philadelphia, Pa

Received for publication October 19, 2007; revisions received August 15, 2008; accepted for publication September 3, 2008.

^_* Address for reprints: Bradley S. Marino, MD, MPP, MSCE, FACC, Associate Professor of Pediatrics, University of Cincinnati College of Medicine, Divisions of Cardiology and Critical Care Medicine, Cincinnati Children's Hospital Medical Center, MLC 2003, 3333 Burnet Ave, Cincinnati, OH 45229. (Email: Bradley.Marino{at}cchmc.org).

	Abstract

Top Abstract Introduction Methods Results Discussion Conclusions Figure E1 Figure E2 Table E1 Table E2 References

 
Objective: In "true" parachute mitral valve, mitral valve chordae insert into one papillary muscle. In parachute-like asymmetric mitral valve, most or all chordal attachments are to one papillary muscle. This study compared morphologic features, associated lesions, and palliation strategies of the two parachute mitral valve and dominant papillary muscle types and examined interventions and midterm outcomes in patients with biventricular circulation.

Methods: Echocardiography and autopsy databases were reviewed to identify patients with "true" parachute mitral valve or parachute-like asymmetric mitral valve from January 1987 to January 2006. Predictors of palliation strategy in the entire cohort, mitral stenosis on initial echocardiogram, and mortality in the biventricular cohort were determined with logistic regression.

Results: Eighty-six patients with "true" parachute mitral valve (n = 49) or parachute-like asymmetric mitral valve (n = 37) were identified. Chordal attachments to the posteromedial papillary muscle were more common (73%). The presence "true" parachute mitral valve (P = .008), hypoplastic left ventricle (P < .001), and two or more left-sided obstructive lesions (P = .002) predicted univentricular palliation. Among 49 patients maintaining biventricular circulation at follow-up, 8 died median follow-up 6.4 years (7 days–17.8 years). Multivariate analysis revealed that "true" parachute mitral valve was associated with mitral stenosis on initial echocardiogram (P = .03), and "true" parachute mitral valve (P = .04) and conotruncal anomalies (P = .0003) were associated with mortality. Progressive mitral stenosis was found in 11 patients; 2 underwent mitral valve interventions, and 1 died.

Conclusion: Nearly two thirds of this parachute mitral valve cohort underwent biventricular palliation. Some progression of mitral stenosis occurred, although mitral valve intervention was rare. "True" parachute mitral valve was associated with mitral stenosis on initial echocardiogram. "True" parachute mitral valve and conotruncal anomalies were associated with mortality in the biventricular population.

	Introduction

Top Abstract Introduction Methods Results Discussion Conclusions Figure E1 Figure E2 Table E1 Table E2 References

 
Congenital mitral stenosis (MS) results from a variety of anatomic anomalies in the pediatric population, and it is commonly associated with parachute mitral valve (PMV),^1,2 an abnormality in which the mitral valve (MV) chordae insert into a single papillary muscle (anterolateral papillary muscle [ALPM] or posteromedial papillary muscle [PMPM]).³ Parachute-like asymmetric mitral valve (PLAMV)⁴ is a similar anomaly in which chordae are distributed unequally between two identifiable papillary muscles. Usually the dominant papillary muscle is normal and the other is elongated and displaced toward the mitral valve annulus. Although both mitral valve anomalies have predominant unifocalization of chordae, "true" PMV has only one papillary muscle to which all chordae are attached. When both types of PMV occur, the chordae are short and thickened, limiting movement of normal mitral valve leaflets³ and resulting in functional and hemodynamic abnormalities.⁵ Although clinical presentations may be similar, "true" PMV and PLAMV are defined separately, because the valves originate and develop in different ways morphogenetically.⁶

PMV is associated with other cardiac anomalies,⁷ which significantly affect patient outcome.⁸ These associations may determine whether a patient requires univentricular palliation, in which an atrial septectomy is performed, making the tricuspid valve the systemic atrioventricular valve and thus rendering the functional outcome of the PMV less important. In patients with "true" PMV or PLAMV and a biventricular circulation, the degree of MS or mitral regurgitation (MR) is of paramount importance.

Historically, "true" PMV and PLAMV have been grouped together and analyzed as a single lesion. The influence of PMV type and dominant papillary muscle on associated lesions, palliation strategies, and outcomes in patients who maintain a biventricular circulation has not been previously analyzed. The objectives of this study were as follows: (1) to describe and compare the morphologic features and associated lesions of the two PMV and dominant papillary muscle types, (2) to assess for predictors of univentricular palliation in the entire PMV cohort, (3) to describe the interventions and midterm outcomes of patients who maintained biventricular circulation, and (4) to assess for predictors of MS on initial echocardiogram, progressive MS and MR, and mortality in the biventricular cohort.

	Methods

Top Abstract Introduction Methods Results Discussion Conclusions Figure E1 Figure E2 Table E1 Table E2 References

 
Study Design
This study was a retrospective case series including all patients with the diagnosis of "true" PMV or PLAMV from January 1, 1987, to January 1, 2006, at The Children's Hospital of Philadelphia. The study was approved by the institutional review board (IRB #2006-3-4758).

Patient Data
Patients were identified through the echocardiography laboratory database and Cardiac Registry via autopsy specimen at The Children's Hospital of Philadelphia. Demographic information, echocardiography, and surgical and catheter-based intervention data were collected from patient charts, echocardiography laboratory, and cardiology databases at The Children's Hospital of Philadelphia. Most autopsy reports identified through the Cardiac Registry did not specify PMV type or dominant papillary muscle, necessitating specimen examination. If disagreement was noted between echocardiogram and autopsy specimen, autopsy data were used. Patients were separated into univentricular and biventricular categories according to palliation strategy. Patients with biventricular circulation who were unavailable for follow-up were excluded from outcome analysis. Patients with common atrioventricular canal were excluded.

Echocardiographic Data
The images from the initial echocardiogram were reviewed for papillary muscle anatomy, original anatomic diagnoses, associated cardiac lesions, and the presence of MS and MR. When all pertinent echocardiographic information was not present, additional echocardiograms were reviewed to complete data acquisition.

"True" PMV was diagnosed when a mitral valve with unifocal attachment to a single papillary muscle was identified with 2-dimensional echocardiography (Figure 1, A ). PLAMV was diagnosed when two papillary muscles were identified, with one papillary muscle receiving most or all of the chordae from the mitral valve leaflets (Figure 1, B). Normal mitral valve anatomy features bifocal attachment of chordae distributed equally between two distinct papillary muscles (Figure 1, C). These definitions are those described by Oosthoek and colleagues.⁴

View larger version (10K): [in this window] [in a new window]   Figure 1. Mitral valve anatomy. Schematic drawings of true parachute mitral valve, with all chordae attached to a single papillary muscle (A); parachutelike asymmetric mitral valve, with chordae attached to two papillary muscles, one being elongated and displaced toward mitral valve annulus (B); and normal mitral valve, with chordae distributed equally between two distinct papillary muscles (C). Reproduced with permission from: 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. 1997;114:9-15.

Follow-up data and most recent echocardiogram were obtained for patients with a biventricular circulation. Most recent echocardiographic results were obtained before any mitral valve intervention, if applicable. The follow-up echocardiogram and data for biventricular patients no longer followed up at our institution were obtained from each patient's cardiologist with permission. Presence, degree, and progression of MS and MR were assessed. Progressive MS was defined as any increase in mean gradient at least 2 mm Hg, an increase in peak gradient greater than 3 mm Hg, or by an increase from no MS to the presence of MS relative to the initial echocardiogram. Progressive MR was defined by any increase in category of regurgitation relative to initial echocardiogram. Patients with only one echocardiogram before death or mitral valve intervention were excluded from analysis of progressive MS or MR.

Statistical Methods
Nonparametric data were expressed as median and range, and parametric data were expressed as mean ± SD. Dichotomous variables were tabulated.

The Fisher exact test was used to assess for associations between PMV type, dominant papillary muscle, and associated lesions, as well as associations between palliative strategy and PMV type, dominant papillary muscle, associated lesions, and increasing levels of left-sided obstruction. Logistic regression with multiple predictors and backward model selection was then used to identify predictors of palliation strategy.

After the biventricular cohort was identified, associations between PMV type, dominant papillary muscle, and surgical and catheter-based interventions were assessed with the Fisher exact test. Logistic regression was used to assess for predictors of MS at initial echocardiogram. Predictors of mortality in the biventricular group were identified by logistic regression and Cox proportional hazard regression analysis. Kaplan–Meier curves for time to death were computed and plotted, and variables that showed an association with mortality were stratified. The log-rank test was used to assess for differences in survival between stratified groups. Multivariate analysis was not used to predict progressive MS, because only two echocardiograms (initial and most recent) were used for analysis, and the exact time after initial echocardiogram at which the MS progressed was not known. Echocardiographic changes in MS and MR were assessed with the McNemar test for correlated proportions. Statistical analysis was performed with R version 2.2.0 (http://www.r-project.org) and STATA 10.0 (StataCorp LP, College Station, Tex).

A sequential Bonferroni correction (Holm method) was used to correct for multiple comparisons.¹⁰ A P value at least .025 but less than .05 was considered to represent a trend toward significance when multiple comparisons were performed. A P value at least .05 but less than .10 was considered a trend toward significance when performing the McNemar test for correlated proportions.

	Results

Top Abstract Introduction Methods Results Discussion Conclusions Figure E1 Figure E2 Table E1 Table E2 References

 
Patient Population
A total of 86 patients with "true" PMV (n = 49) or PLAMV (n = 37) were identified. Eighty-one patients were identified in the echocardiography database, and 5 additional patients were identified solely in the Cardiac Registry from autopsy reports; all of the latter had univentricular circulation. The patients identified solely in the Cardiac Registry all predated the echocardiography database, and echocardiogram reports were not available for review. Most of the patients (n = 54, 63%) were male, and the median age at initial echocardiogram was 11 days (range 1 day–16.8 years). In 86% of the patients, the initial echocardiogram revealed all the echocardiographic data points, whereas 8% required evaluation of a second echocardiogram. Six percent of the cohort had anatomic details obtained from autopsy specimen inspection. Chordal attachments were more common to the PMPM (n = 63, 73%). One or more associated cardiac lesions were found in 85 of 86 patients and are listed in Table 1 . Sixty-two patients (72%) in the cohort had at least one level of left-sided obstruction, 40 patients (47%) had two or more levels of left-sided obstruction, and 18 patients (21%) had three or more levels of left-sided obstruction. Eleven patients (12%) had conotruncal anomalies (interrupted aortic arch, tetralogy of Fallot, double-outlet right ventricle, dextrotransposition of the great arteries) and had right and/or left-sided obstructive lesions. Associated genetic defects, noncardiac anomalies, or syndromes were found in 13 patients (15%) and are described in Table E1; the most commonly identified noncardiac abnormality was 22q11 microdeletion (n = 4, 4.7%).

Management
Three patients in the cohort (3.5%) did not undergo any surgical or catheter-based intervention and proceeded down a biventricular management pathway. Biventricular palliation was pursued in 58.1% of the patients (n = 50), and univentricular palliation was undertaken in 38.4% (n = 33). Patient demographic characteristics and associated cardiac lesions by palliation strategy are noted in Table 2 . There was an association between an increasing number of levels of left-sided obstruction and univentricular palliation (P < .001). When stratifying by number of levels of obstruction, the presence of two or more levels of left-sided obstruction was strongly associated with univentricular palliation (P < .001). One level of left-sided obstruction was not statistically significantly associated with univentricular palliation.

Most patients with "true" PMV and PLAMV who underwent univentricular palliation had a Norwood procedure performed as the initial operation (n = 31, 94%). Two patients had pulmonary artery banding performed at the neonatal palliation. Both of these patients later underwent a hemi-Fontan procedure in combination with a Damus–Kaye–Stansel procedure. Initial neonatal palliation procedures for the univentricular group are noted in Table 3 .

Two patients (2/49, 4%) underwent a total of 3 mitral valve surgeries or catheter-based interventions. One patient underwent mitral valvotomy; the other underwent mitral valvotomy followed by mitral valve replacement and died 4 months later. Too few patients underwent mitral valve intervention to analyze for predictors of MV intervention.

Biventricular Group Follow-up
Echocardiographic data
Of the 49 patients in the biventricular follow-up group, 1 (2%) had a single echocardiogram before death and was therefore not included in MS or MR analysis because progressive MS and progressive MR analysis required serial echocardiograms. The 48 remaining patients had a median follow-up of 6.4 years (7 days–17.8 years).

Sixteen (33%) patients had MS at initial echocardiogram (Figures 2 and E1). The initial median peak gradient in patients with MS was 14 mm Hg (6–20 mm Hg), with a median mean gradient of 6 mm Hg (5–13 mm Hg). On the most recent echocardiogram, 22 of the patients in the biventricular group (46%) had MS. The median peak gradient at follow-up was 15 mm Hg (9–29 mm Hg), with a median mean gradient of 6 mm Hg (4–14 mmHg). Eleven of the 22 patients (50%) had progressive MS. Although there was a trend toward progression of MS from initial to most recent echocardiogram (P = .07), 5 of 16 patients with initial MS had a decrease in level of MS from initial to most recent echocardiogram. Two of the 5 patients with a decrease in MS from initial to most recent echocardiogram had undergone ventricular septal defect closure during the interim period.

View larger version (14K): [in this window] [in a new window]   Figure 2. Mitral stenosis and mitral regurgitation at initial and most recent echocardiograms in patients with biventricular circulation. Percentages of patients in biventricular cohort with mitral stenosis and mitral regurgitation at initial and most recent echocardiogram.

Predictors of mortality
Within the biventricular cohort, 8 patients (16%) died (Table E2). Three of these 8 patients were known to have a primary left-sided obstructive lesion and died as a direct result of the consequences of long-standing moderate or greater MS and pulmonary hypertension. One of the patients had pulmonary hemorrhage that was worsened by moderate MS and elevated pulmonary arterial pressures. Two died of respiratory failure unrelated to the presence of the PMV. Mortality did not statistically differ by sex, presence of MS or MR at initial echocardiogram, or presence of progressive MS or MR at most recent echocardiogram.

Among the 8 patients who died in the biventricular group, 7 patients (88%) had "true" PMV, whereas 1 had PLAMV. Mortality was associated with having "true" PMV (P = .02) but was not statistically significantly related to a dominant papillary muscle. All 8 patients who died had a ventricular septal defect, and 6 of 8 had a conotruncal anomaly (tetralogy of Fallot, interrupted aortic arch, aortic arch hypoplasia with ventricular septal defect); both malalignment ventricular septal defect (P = .004) and conotruncal anomaly (P < .001) were associated with mortality by univariate analysis. In addition, surgical repair of ventricular septal defect was associated with mortality (P = .02).

Freedoms from mortality for the entire biventricular cohort at 1, 5, and 10 years after initial diagnosis are shown in Figure 3 (A). The 1-, 5-, and 10-year survivals after stratification by "true" PMV and conotruncal anomalies are plotted in Figure 3 (B and C). After stratification, the log-rank test for the Kaplan–Meier curves was significant for both "true" PMV (P = .02) and conotruncal anomaly (P < .001). When Cox proportional hazard regression analysis was used, only conotruncal anomaly was a significant predictor of mortality (P = .002).

View larger version (13K): [in this window] [in a new window]   Figure 3. Kaplan–Meier survival curves. Freedoms from mortality for entire biventricular cohort (A), and stratified for parachute mitral valve type (B) and presence of conotruncal anomaly (C).

	Discussion

Top Abstract Introduction Methods Results Discussion Conclusions Figure E1 Figure E2 Table E1 Table E2 References

 
In the past, "true" PMV and PLAMV have been grouped together under the single anomaly of PMV. This study further confirms that PMV is not an isolated cardiac anomaly. Only 1 patient in the cohort had PMV without an associated cardiac defect. The most commonly associated cardiac lesions on initial echocardiogram were patent ductus arteriosus, coarctation of the aorta, and ventricular septal defect. As in other studies describing the lesions associated with PMV, left-sided obstructive cardiac lesions and ventricular septal defects were common in our cohort.^{3-5,7-8,11-13} There was no association noted between the number of levels of obstruction and PMV type. Interestingly, conotruncal anomalies accounted for 13% of our PMV cohort. Similarly, Tandon and associates¹³ reported that 26% of the specimens in a pathologic analysis of 52 cases of PMV had conotruncal anomalies.

To our knowledge, this is the first study to examine associated cardiac lesions by PMV type and dominant papillary muscle, although no statistically significant associations were noted. There was no association between PMV type or dominant papillary muscle and hypoplastic left ventricle, aortic stenosis or atresia, or subaortic stenosis as single entities. This may have resulted from the smaller sample size used for subgroup analyses.

Because "true" PMV and PLAMV are not isolated lesions, it is not surprising that the vast majority of patients underwent at least one surgical or catheter-based intervention separate from the PMV. We believe this to be the first study to evaluate palliation strategy according to PMV type and dominant papillary muscle. Univentricular palliation was elected in 38% of patients. Almost three fourths of the patients in our study who required univentricular palliation had "true" PMV. In multivariate analysis, "true" PMV was associated with univentricular palliation, along with hypoplastic left ventricle and the presence of two or more levels of left-sided obstruction. We speculate that "true" PMV may result in reduced left ventricular inflow relative to PLAMV and may result in left ventricular hypoplasia, aortic stenosis, arch hypoplasia, or all three. Excluding patients in which support was withdrawn, Schaverien and coworkers⁸ showed that 13% of their patients underwent univentricular palliation, but they did not differentiate by PMV type or dominant papillary muscle.

Among patients who underwent biventricular palliation, patients with "true" PMV had a greater risk for mortality than did patients with PLAMV. Mortality in the biventricular cohort was also associated with conotruncal anomaly. Of the 8 patients who died in the biventricular group, 6 (75%) had conotruncal anomaly, and 5 of these 6 had "true" PMV. This differs considerably from the findings of Oosthoek and associates,⁴ whose patients with conotruncal anomaly had PLAMV almost exclusively. Both the higher incidence of conotruncal anomaly in Oosthoek and associates' autopsy cohort⁴ and the deaths in our study cohort may potentially have resulted from right- and left-sided obstructive disease. Schaverien and coworkers⁸ found the presence of hypoplastic left ventricle and atrial septal defect to be associated with mortality in patients with PMV. The mortality analysis performed by Schaverien and coworkers⁸ included patients from their PMV cohort both biventricular and univentricular palliation. Because atrial septal defect and left ventricular hypoplasia are markers for severe left-sided heart obstruction, which would likely be associated with univentricular palliation or higher-risk biventricular palliation, it is not surprising that these markers were associated with death. All patients in our cohort who had severe left-sided obstruction underwent univentricular palliation and thus were not included in our biventricular mortality analysis.

In our biventricular cohort, we found a significant association between "true" PMV and the presence of MS at initial echocardiogram according to multivariate analysis. Although we found a trend toward progressive MS from initial to most recent echocardiogram in our study, the analysis was complicated because the group had both progression and regression. In the vast majority of cases in which MS progressed, MS was not noted at initial echocardiogram. Among those patients with MS on initial echocardiogram, most remained at the same level from initial to follow-up echocardiogram. In the minority of patients in whom the gradient of MS fell from initial to most recent echocardiogram, ventricular septal defect closure had occurred in the interim, thereby reducing the preload on the left atrium.

In our study, we found a significant increase in MR from initial to most recent echocardiogram. Of note, most patients with progressive MR did not have MR at initial echocardiogram, and only a small number of patients had progression to hemodynamically significant MR. Despite the rarity of patients with moderate or greater MR at most recent echocardiogram, we believe that patients with PMV may be at risk for progressive MR and should be followed up long-term for hemodynamically significant MR.

Only 4% of the patients in our biventricular cohort (2/49) underwent mitral valve interventions. This incidence is lower than that reported by Schaverien and coworkers,⁸ who reported mitral valve intervention in 19% of their cohort. This may have been because more patients underwent univentricular palliation in our cohort, or there may have been a difference in institutional biases regarding when to perform mitral valve intervention for MS.

Study Limitations
This study has several potential limitations. It is a retrospective study, and therefore information bias may exist. The follow-up period between first and last echocardiogram differed between patients and may not have provided the long-term follow-up required to assess the effects on mortality of PMV type, dominant papillary muscle, associated cardiac lesions, multiple levels of left-sided obstruction, and progressive MS and MR. Because of the prevalence of ventricular septal defect and patent ductus arteriosus as associated lesions, some patients may have had the gradient across the mitral valve overestimated as a consequence of an increased left atrial preload. Additionally, the prevalence of ventricular septal defect as an associated lesion may have affected the ability to assess reliably obstructive lesions of the aortic valve or aortic arch in some patients because of left-to-right shunting at the ventricular level. Because of different biases toward univentricular and biventricular palliation strategies among centers, the patients in our biventricular cohort may differ from other biventricular cohorts at other centers, where more patients might undergo biventricular palliation and therefore undergo more frequent mitral valve interventions. Although all echocardiograms at our institution were reviewed by a single cardiologist for anatomy and associated lesions, follow-up data for patients no longer at our institution were obtained from outside echocardiogram reports. Additionally, only 2 echocardiograms per patient were reviewed, which may have limited the sensitivity of the progressive MS and MR analyses.

	Conclusions

Top Abstract Introduction Methods Results Discussion Conclusions Figure E1 Figure E2 Table E1 Table E2 References

 
Both "true" PMV and PLAMV have a broad spectrum of associated lesions, both of which have PMPM as the dominant papillary muscle. "True" PMV, hypoplastic left ventricle, and the presence of two or more left-sided obstructive lesions were predictors of univentricular palliation in our center, and in patients with biventricular palliation, "true" PMV and conotruncal anomalies were associated with mortality. At midterm follow-up, among patients who underwent biventricular palliation, progressive MS as seen on echocardiogram was not uncommon, but mitral valve intervention was rare. MR in this group was progressive but hemodynamically insignificant. Although progressive MS has not been associated with mitral valve intervention or mortality in our study to date, progression of MS should be followed carefully in patients with "true" PMV and PLAMV in this high-risk population.

	Figure E1

Top Abstract Introduction Methods Results Discussion Conclusions Figure E1 Figure E2 Table E1 Table E2 References

 

Progression of mitral stenosis in patients with biventricular circulation. Mitral stenosis status at initial and most recent echocardiogram (Echo).

	Figure E2

Top Abstract Introduction Methods Results Discussion Conclusions Figure E1 Figure E2 Table E1 Table E2 References

 

Progression of mitral regurgitation in patients with biventricular circulation. Mitral regurgitation status at initial and most recent echocardiogram (Echo).

	Table E1

Top Abstract Introduction Methods Results Discussion Conclusions Figure E1 Figure E2 Table E1 Table E2 References

 

Associated noncardiac abnormalities

No. % 22q11 Microdeletion disorders 4 4.7% 9p Syndrome mosaic 1 1.2% Trisomy 21 mosaic 1 1.2% Turner mosaic 1 1.2% Trisomy 18 1 1.2% Trisomy 8 1 1.2% Partial trisomy 6 1 1.2% 6q Deletion 1 1.2% Jobert syndrome 1 1.2% Goldenhar syndrome 1 1.2% Noonan syndrome 1 1.2% Microcephaly 1 1.2% Dysplastic kidneys 1 1.2% Duodenal atresia 1 1.2% Hemorrhagic gastritis 1 1.2% Malrotation of intestine 1 1.2%

	Table E2

Top Abstract Introduction Methods Results Discussion Conclusions Figure E1 Figure E2 Table E1 Table E2 References

 

Mortality in the biventricular group

Patient PMV type Dominant PM Original diagnosis Procedures Cause of death 1 PLAMV ALPM TOF TOF repair, PDA ligation Unknown 2 True PMV ALPM Arch hypoplasia, VSD Coarctation repair, VSD repair Pulmonary hypertension 3 True PMV PMPM TOF and PA with MAPCAs Unifocalization, RV–pulmonary artery conduit, LPA/RPA balloon dilation angioplasty and stenting, resection of RV pseudoaneurysm, coil embolization of collaterals Pulmonary hemorrhage followed by cardiac arrest 4 True PMV ALPM MS, VSD VSD repair, mitral valvuloplasty, MVR Pulmonary hypertension 5 True PMV PMPM TOF and PA with confluent pulmonary arteries RV to pulmonary artery conduit placement, VSD closure Unknown 6 True PMV PMPM Trisomy 18, VSD, ASD, PDA, dysplastic PV No procedures Respiratory failure 7 True PMV PMPM IAA Aortic arch repair, PAB (x2), debanding, VSD closure, residual VSD closure, RVOT reconstruction, arch patch augmentation for recoarctation, pacemaker for resynchronization therapy DCM, cardiac arrest while awaiting heart transplant 8 True PMV PMPM TOF with absent PV TOF repair with plication of pulmonary arteries Respiratory failure

PMV, Parachute mitral valve; PM, papillary muscle; PLAMV, parachutelike asymmetric mitral valve; ALPM, anterolateral papillary muscle; TOF, Tetralogy of Fallot; PDA, patent ductus arteriosus; VSD, ventricular septal defect; PMPM, posteromedial papillary muscle; PA, pulmonary atresia; MAPCA, major aortopulmonary collateral artery; RV, right ventricle; LPA, left pulmonary artery; RPA, right pulmonary artery; MS, mitral stenosis; MVR, mitral valve replacement; ASD, atrial septal defect; PV, pulmonary valve; IAA, interrupted aortic arch; PAB, pulmonary arterial banding; RVOT, right ventricular outflow tract; DCM, dilated cardiomyopathy.

	Footnotes

 
^_* These authors share credit as second authors.

	References

Top Abstract Introduction Methods Results Discussion Conclusions Figure E1 Figure E2 Table E1 Table E2 References

 

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