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Right coronary cusp prolapse resembling subpulmonic stenosis in an old adult patient with ventricular septal defect.
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MedLine Citation:
PMID:  22259668     Owner:  NLM     Status:  PubMed-not-MEDLINE    
Abstract/OtherAbstract:
Ventricular septal defect (VSD) can be associated with various complications such as aortic regurgitation (AR). AR in VSD come from a deficiency or hypoplasia of the conal septum which leads to abnormal apposition in diastole and prolapse of the poorly supported noncoronary or right coronary cusp through the VSD into the right ventricle resembling subpulmonic stenosis and subsequently results in distortion of the aortic valve and progressive AR. AR often increases in severity with age and it indicates a worse prognosis. Therefore, appropriate timing of surgical repair in progressive AR in VSD might be important. Until now, many earlier experiences about surgical repair of AR complicating VSD were on adolescents or young adults. We reported a case of AR in 48-year-old male patient with right coronary cusp prolapse complicating the subarterial type of VSD which was properly assessed by echocardiography and was successfully treated with surgical repair. Right coronary cusp or noncoronary cusp prolapse should be suspected in AR complicating VSD through proper echocardiographic assessment and the surgical repair on VSD and distorted aortic valve should be considered in the old patient, as well as the young.
Authors:
Myeong Gun Kim; Wook-Jin Chung; Chang Hyu Choi; Jeonggeun Moon; Mi-Seung Shin; Seung Hwan Han; Eak Kyun Shin
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Publication Detail:
Type:  Journal Article     Date:  2011-12-27
Journal Detail:
Title:  Journal of cardiovascular ultrasound     Volume:  19     ISSN:  2005-9655     ISO Abbreviation:  J Cardiovasc Ultrasound     Publication Date:  2011 Dec 
Date Detail:
Created Date:  2012-01-19     Completed Date:  2012-10-02     Revised Date:  2013-05-29    
Medline Journal Info:
Nlm Unique ID:  101477138     Medline TA:  J Cardiovasc Ultrasound     Country:  Korea (South)    
Other Details:
Languages:  eng     Pagination:  216-20     Citation Subset:  -    
Affiliation:
Cardiology Division, Heart Center, Gachon University Gil Hospital, Incheon, Korea.
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Full Text
Journal Information
Journal ID (nlm-ta): J Cardiovasc Ultrasound
Journal ID (publisher-id): JCU
ISSN: 1975-4612
ISSN: 2005-9655
Publisher: Korean Society of Echocardiography
Article Information
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Copyright © 2011 Korean Society of Echocardiography
open-access:
Received Day: 08 Month: 8 Year: 2011
Revision Received Day: 23 Month: 11 Year: 2011
Accepted Day: 30 Month: 11 Year: 2011
Print publication date: Month: 12 Year: 2011
Electronic publication date: Day: 27 Month: 12 Year: 2011
Volume: 19 Issue: 4
First Page: 216 Last Page: 220
ID: 3259549
PubMed Id: 22259668
DOI: 10.4250/jcu.2011.19.4.216

Right Coronary Cusp Prolapse Resembling Subpulmonic Stenosis in an Old Adult Patient with Ventricular Septal Defect
Myeong Gun Kim, MD13
Wook-Jin Chung, MD13
Chang Hyu Choi, MD23
Jeonggeun Moon, MD13
Mi-Seung Shin, MD13
Seung Hwan Han, MD13
Eak Kyun Shin, MD13
1Cardiology Division, Heart Center, Gachon University Gil Hospital, Incheon, Korea.
2Department of Cardiovascular Surgery, Gachon University Gil Hospital, Incheon, Korea.
3Gachon Cardiovascular Research Institute, Gachon University School of Medicine, Incheon, Korea.
Correspondence: Address for Correspondence: Wook-Jin Chung, Cardiology Division, Heart Center, Gachon University Gil Hospital, 1198 Guwol-dong, Namdong-gu, Incheon 405-760, Korea. Tel: +82-32-460-3054, Fax: +82-32-460-1906, wjcheart@gmail.com

Introduction

Ventricular septal defect (VSD) accounts for 20% of the congenital cardiovascular malformations and 10% of those VSDs diagnosed in adults. The vast majority of VSDs (roughly 70%) are located in the area of the membranous septum and they are defined as being perimembranous or subarterial. With a relative high incidence in Asian countries,1) the association of the subarterial type VSD with aortic valve prolapse (AVP), and mainly right coronary cusp prolapse, and aortic regurgitation (AR) has been shown. According to the previous reports, the incidence of AVP in the subarterial VSD was 36% to 79%.2), 3) The peak age for AVP is around 7 years, and that for AR is between 5 and 10 years.3), 4) VSD is a very common congenital heart defect in children, but due to spontaneous and surgical closure, it is less commonly encountered in adults.

When VSD is found in adult, about one-third of patents with VSD initially managed medically required surgical intervention later in life.5-7) In a tertiary referral population, there was an report that 25% of patients with small VSDs had serious complications, such as AR and endocarditis; these were the most common indications for surgical VSD closure in adults.6) Many experiences published case reports about surgical repair of AR complicating VSD in adolescents or young adults patients.5-7) But old adult patient was little known.

Here we reported a case of AR in a patient with right coronary cusp prolapse that was complicating a subarterial type VSD and the patient was 48 years old. It was diagnosed by thorough echocardiographic assessment and the patient underwent successful surgical closure of the VSD along with aortic valvuloplasty.


Case

A 48-year-old male visited emergency department due to retrosternal chest pain at rest for 6 hours and dyspnea on exertion for 3 months. He had been diagnosed with hypertension a year previously, but he had not taken any medication. He was a current smoker with 30 pack-years history and he drank alcohol daily. However, he had no medical history of arrhythmias, diabetes mellitus, ischemic heart disease or stroke. On arrival in the emergency department, his blood pressure was 150/90 mm Hg, his pulse rate was 100 bpm, his respiratory rate was 18 breaths per minute and his body temperature was 36.5℃. Cardiac auscultation revealed regular heart sounds with a pansystolic murmur and the lung sounds were clear. The initial electrocardiogram showed normal sinus rhythm and the chest radiography demonstrated a normal sized heart. The laboratory studies were within the normal limits.

The initial transthoracic echocardiogram (TTE) revealed mild right ventricular outflow obstruction resembling subpul monic stenosis (Fig. 1), a subarterial type VSD of 4.2 mm diameter with left to right shunt flow (Qp/Qs = 2.01) and pressure gradient corresponding to 136 mm Hg. The right ventricular systolic pressure was 69 mm Hg and right ventricle was mildly hypertrophic. In parasternal long-axis view of TTE, the high velocity jet produced by the left-to-right shunt, through the VSD, results in a Venturi effect, displacing the unsupported annulus outward into the right ventricle (Fig. 2A and B). There was severe AR (Fig. 2C and D) with subtle right coronary cusp prolapse which caused mild right ventricular outflow obstruction. Also, in parasternal short-axis view at aortic level of TTE, there was right ventricular outflow tract obstruction due to right coronary cusp (RCC) driven into the right ventricle by high velocity left-to-right shunt flow (Fig. 3A and B).

Cardiac catheterization revealed that Qp/Qs and mean pulmonary artery pressure were measured as 1.41 and 19 mm Hg, respectively. Coronary angiography showed no significant luminal narrowing in coronary arteries.

Although the Qp/Qs was low, we decided to perform an operation because he had dyspnea and severe AR. The surgical findings showed a prolapsed and retracted RCC and commissural calcification between the RCC and the noncoronary cusp (NCC). The VSD was nearly closed by the RCC (Fig. 4). He underwent surgical VSD closure with autopericardium through a pulmonary arteriotomy, and RCC mobilization with decalcification and commissurotomy between the RCC and the NCC. The post-operative course was uneventful. Post-operative TTE was performed and this revealed no residual shunt and only mild AR. In follow-up (after 48 months) examination, he had no symptom, and there was no specific change in follow-up TTE.


Discussion

In this report, we present a case of complicated VSD with severe AR due to right coronary cusp prolapse resembling subpulmonic stenosis in the relatively old adult. Although the patient's complicated VSD was diagnosed later in life, he was successfully treated with surgical repair after thorough echocardiographic assessment.

Echocardiography is the noninvasive method of choice for evaluating a VSD. It is a sensitive, descriptive tool with an excellent detection rate (88% to 95%), depending on the size and location of the defect and the technician's experience.8), 9)

Thorough echocardiographic examination is best achieved by imaging the interventricular septum in multiple planes using color flow and spectral Doppler echocardiography. Echocardiography is most sensitive for defects larger than 5 mm and that are located in the membranous, inlet or outlet portion of the septum. It is least sensitive for detecting apical muscular defects. Echocardiography can confidently identify the morphologic features of the defect, including its size and borders and any associated defects.10), 11) It also provides accurate hemodynamic assessment of the shunt and its severity, the volume overload, a subpulmonic (double-chambered right ventricle) or pulmonic stenosis, and pulmonary hypertension.12) In addition, echocardiography can assess the degree of aortic valve distortion and prolapse (the right and noncoronary cusps) in patients with a subarterial VSD and it can evaluate the severity of AR and right ventricular outflow tract obstruction caused by the prolapsing coronary cusp.13) Yearly echocardiographic assessment of AR has important prognostic implications, and especially with regard to the timing of surgical treatment. The mechanism of AVP in patients with subarterial VSD may include a lack of infundibular septal support, intrinsic discontinuity of the aortic valve annulus and aortic media, and a Venturi effect of the VSD jet.4)

The risk for AVP and regurgitation in patients with membranous or subarterial defects increases with age.14), 15) AR is believed to be 2.5 times more frequent in patients with subarterial VSDs. Momma et al.15) reported on 395 patients with subarterial defects: AR was seen in 50% of the patients by 8 years and in 87% of the patients by 20 years. As the AVP progresses, the intraventricular shunting decreases at the expense of aortic valve distortion and regurgitation. This increases the risk for endocarditis, left ventricular volume overload, and less commonly, right ventricular outflow tract obstruction and sinus of Valsalva aneurysm.16), 17) The progressive nature of the AR and the associated increased morbidity have led to recommending early surgical intervention.18), 19) However, unless endocarditis supervenes, the rate of progression is often slow and variable. Therefore, the proper timing and type of operation are still controversial. The treatment of an isolated VSD depends on the type of defect, its size, the shunt's severity, the pulmonary vascular resistance, the functional capacity and the associated acquired anomalies, such as AR, subpulmonic stenosis, or pulmonary hypertension.20), 21) Surgical closure of a VSD in adult decreases the risk for endocarditis by at least 50%, it reduces the pulmonary artery pressure, it improves the functional classification.21), 22) and it can be accomplished with very low short- and long-term mortality and morbidity.23)

The case gives us two important lessons. The first, RCC or NCC prolapse should be suspected in AR complicating VSD through proper echocardiographic assessment. The second lesson was surgical repair of complicated VSD provides good result to the old adult patient, as well as the young.


References
1. Ammash NM,Warnes CA. Ventricular septal defects in adultsAnn Intern MedYear: 200113581282411694106
2. Soto B,Becker AE,Moulaert AJ,Lie JT,Anderson RH. Classification of ventricular septal defectsBr Heart JYear: 1980433323437437181
3. Ueda M,Fujimoto T,Becker AE. The infundibular septum in normal hearts and in hearts with isolated ventricular septal defect. A comparison between Japanese and Dutch heartsJpn Heart JYear: 1986276356433820574
4. Elgamal MA,Hakimi M,Lyons JM,Walters HL 3rd. Risk factors for failure of aortic valvuloplasty in aortic insufficiency with ventricular septal defectAnn Thorac SurgYear: 1999681350135510543505
5. Kidd L,Driscoll DJ,Gersony WM,Hayes CJ,Keane JF,O'Fallon WM,Pieroni DR,Wolfe RR,Weidman WH. Second natural history study of congenital heart defects. Results of treatment of patients with ventricular septal defectsCirculationYear: 199387I38I518425321
6. Neumayer U,Stone S,Somerville J. Small ventricular septal defects in adultsEur Heart JYear: 199819157315829820997
7. Gabriel HM,Heger M,Innerhofer P,Zehetgruber M,Mundigler G,Wimmer M,Maurer G,Baumgartner H. Long-term outcome of patients with ventricular septal defect considered not to require surgical closure during childhoodJ Am Coll CardiolYear: 2002391066107111897452
8. Lue HC,Sung TC,Hou SH,Wu MH,Cheng SJ,Chu SH,Hung CR. Ventricular septal defect in Chinese with aortic valve prolapse and aortic regurgitationHeart VesselsYear: 198621111163759798
9. Mori K,Matsuoka S,Tatara K,Hayabuchi Y,Nii M,Kuroda Y. Echocardiographic evaluation of the development of aortic valve prolapse in supracristal ventricular septal defectEur J PediatrYear: 19951541761817758512
10. Sohn DW. Symptom based echocardiographic approach: dyspneaJ Korean Soc EchocardiogrYear: 20041259
11. Pieroni DR,Nishimura RA,Bierman FZ,Colan SD,Kaufman S,Sanders SP,Seward JB,Tajik AJ,Wiggins JW,Zahka KG. Second natural history study of congenital heart defects. Ventricular septal defect: echocardiographyCirculationYear: 199387I80I888425326
12. Garg A,Shrivastava S,Radhakrishnan S,Dev V,Saxena A. Doppler assessment of interventricular pressure gradient across isolated ventricular septal defectClin CardiolYear: 1990137177212257713
13. Schmidt KG,Cassidy SC,Silverman NH,Stanger P. Doubly committed subarterial ventricular septal defects: echocardiographic features and surgical implicationsJ Am Coll CardiolYear: 198812153815463057035
14. Backer CL,Idriss FS,Zales VR,Ilbawi MN,DeLeon SY,Muster AJ,Mavroudis C. Surgical management of the conal (supracristal) ventricular septal defectJ Thorac Cardiovasc SurgYear: 1991102288295 discussion 295-6. 1865702
15. Momma K,Toyama K,Takao A,Ando M,Nakazawa M,Hirosawa K,Imai Y. Natural history of subarterial infundibular ventricular septal defectAm Heart JYear: 1984108131213176496290
16. Mahoney LT. Acyanotic congenital heart disease. Atrial and ventricular septal defects, atrioventricular canal, patent ductus arteriosus, pulmonic stenosisCardiol ClinYear: 1993116036168252562
17. Menahem S,Johns JA,del Torso S,Goh TH,Venables AW. Evaluation of aortic valve prolapse in ventricular septal defectBr Heart JYear: 1986562422493756041
18. Leung MP,Beerman LB,Siewers RD,Bahnson HT,Zuberbuhler JR. Long-term follow-up after aortic valvuloplasty and defect closure in ventricular septal defect with aortic regurgitationAm J CardiolYear: 1987608908943661405
19. Otterstad JE,Erikssen J,Frøysaker T,Simonsen S. Long term results after operative treatment of isolated ventricular septal defect in adolescents and adultsActa Med Scand SupplYear: 19867081393461690
20. Ellis JH 4th,Moodie DS,Sterba R,Gill CC. Ventricular septal defect in the adult: natural and unnatural historyAm Heart JYear: 19871141151203604855
21. Gersony WM,Hayes CJ,Driscoll DJ,Keane JF,Kidd L,O'Fallon WM,Pieroni DR,Wolfe RR,Weidman WH. Second natural history study of congenital heart defects. Quality of life of patients with aortic stenosis, pulmonary stenosis, or ventricular septal defectCirculationYear: 199387I52I658425323
22. Warnes CA,Williams RG,Bashore TM,Child JS,Connolly HM,Dearani JA,del Nido P,Fasules JW,Graham TP Jr,Hijazi ZM,Hunt SA,King ME,Landzberg MJ,Miner PD,Radford MJ,Walsh EP,Webb GD,Smith SC Jr,Jacobs AK,Adams CD,Anderson JL,Antman EM,Buller CE,Creager MA,Ettinger SM,Halperin JL,Hunt SA,Krumholz HM,Kushner FG,Lytle BW,Nishimura RA,Page RL,Riegel B,Tarkington LG,Yancy CW. American College of CardiologyAmerican Heart Association Task Force on Practice Guidelines (Writing Committee to Develop Guidelines on the Management of Adults With Congenital Heart Disease)American Society of EchocardiographyHeart Rhythm SocietyInternational Society for Adult Congenital Heart DiseaseSociety for Cardiovascular Angiography and InterventionsSociety of Thoracic SurgeonsACC/AHA 2008 guidelines for the management of adults with congenital heart disease: a report of the American College of Cardiology/American Heart Association Task Force on Practice Guidelines (Writing Committee to Develop Guidelines on the Management of Adults With Congenital Heart Disease). Developed in Collaboration With the American Society of Echocardiography, Heart Rhythm Society, International Society for Adult Congenital Heart Disease, Society for Cardiovascular Angiography and Interventions, and Society of Thoracic SurgeonsJ Am Coll CardiolYear: 200852e1e12119038677
23. Mongeon FP,Burkhart HM,Ammash NM,Dearani JA,Li Z,Warnes CA,Connolly HM. Indications and outcomes of surgical closure of ventricular septal defect in adultsJACC Cardiovasc IntervYear: 2010329029720298987

Article Categories:
  • Case Report

Keywords: Ventricular septal defect, Aortic valve, Aortic regurgitation, Prolapse.

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