Rosa Nanini 1

Isabella Rosa Nanini

Mr. Speice

Independent Study Mentorship II 3B

18 November 2018

Background Knowledge of Congenital Mitral Valve Malformation, Mitral Valvuloplasty, Mitral

Valve Replacements, and Medical Research

Overview

Being only a high school student my overall knowledge of pediatric cardiothoracic

surgery can become quite limited. The goal of this original work research is to create the

background knowledge needed for my final product a research titled ​Mitral Valvuloplasty and

Mitral Valve Replacements in Infants Less Than One Year Old​. This research will become a

handbook to my understanding of the more detailed prospective research and will answer

questions that may arise during this research.

Mitral Valve Anatomy

The mitral valve is located between the left ventricle and left atrium and it allows blood

flow from the left ventricle to the left atrium. The left side of the heart pumps blood to the aorta

which allows blood to flow the vital organs in the body and the body in general. Highlighting the

importance of a properly working mitral valve. The mitral valve consists of the annulus which is

C shaped like a saddle. The annulus consists of two leaflets the anterior leaftet (A1, A2, and A3)

and the posterior leaflet (P1, P2, and P3). Between the anterior and posterior leaflets the

anterolateral line of commissure and posteromedial line of commissure can be found. The mitral

valve also consists of the subvalvular apparatus (chordae tendineae and papillary muscles) which
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secure the annulus and allows for the opening and closing of the mitral valve. The mitral valve

has no attachment to the septum and during systole when the leaflets meet to close the ventricle,

the line of commisure curves and forms a “smile”. The chordae tendineae connects the leaflet

anatomy to two ventricular papillary muscles.

Congenital Mitral Valve Malformations

Mitral valve prolapse occurs when the leaflets extend above the plane of the mitral

annulus during ventricular systole. This is the most common cardiac vascular anomaly; however,

its uncommon before adolescence. Treatment usually includes mitral valve repair/annuloplasty

for several mitral regurgitation or ventricular enlargement. Although a common malformation,

this malformation will probably not relate to our research since its uncommonly seen in infants

or even children. The next discussed malformation was an isolated cleft, a rare malformation,

which is division of one of the leaflets and is divided within two groups with normal great

arteries and with abnormal conus. When suffering from severe regurgitation mitral valve

replacement patients need to be operated early in life, which means this malformation will

possibly be related with our research. Next is a double orifice mitral valve which is extremely

rare and seen only in 1% of autopsies of congenital heart defects and is rarely isolated and

found within more complex cardiac malformations. Double orifice mitral valves contain a single

fibrous annulus with two orifices opening into the left ventricle. It is divided by three types the

incomplete bridge, the complete bridge, and hole type. Isolated double orifice malformations

require no repair, but when combined with other malformations surgery is often performed. This

malformation is probably not related to our research since this defect is rarely found isolated and

does not require surgery. A mitral ring was also briefly talked about, this an extremely rare
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defect divided into two parts the supramitral ring which is a fibrous membrane originating above

the mitral annulus and the intra-mitral ring with is located beneath the orifice of the left atrial

appendage. This defect is exceptionally isolated and is often combined with various other defects

like the double orifice mitral valve. Being non isolated this malformation will probably be

unrelated to our research. The next types of malformations relate to the tensor apparatus starting

with an arcade/hammock valve. In this malformation the valve mimics a hammock when

observed from the surgeon’s perspective and happens when the tendinous cords are thickened

and short, which leads to abnormal leaflet excursion which may cause stenosis or insufficiency.

When not severe this malformation may have a late discovery and a surgical repair will happen

later in life. I believe this malformation will be unrelated to my research.

Congenital Mitral Valve Malformation Management

Often a surgery or intervention is rarely needed before the first year of life regarding

mitral regurgitation; however, regarding mitral stenosis may require early surgery which is

important to note when leading into more research for my publication. In age less than one often

hammock mitral valve and associated cardiac anomalies are seen. Regarding malformations of

the leaflets, chordae tendineae, and papillary muscles, early surgery is extremely important

which highlights the importance of our research. Mitral valve repairs are also often

recommended over replacements due to difficulty of living with an artificial valve that will grow

with the patient.

The topic of mitral valve malformation management is the prime reason of the purpose of

our research. There is a lack of information of the result of malformation regarding infants less

than one year old, so this aspect of my original work is less researched upon.
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Chart Review in Pediatric Cardiothoracic Surgery (More specific to mitral valve surgery)

Mitral Valve Gradient: The mitral valve gradient can be measured through an

echocardiogram by tracing the outline of mitral diastolic inflow and the mean pressure gradient

will be automatically calculated. A normal mean pressure is < 2mmHg; however, a patient with a

congenital mitral valve malformation will most likely have mitral stenosis which is characterized

with a mean pressure gradient of 5mmHg to 10mmHg for moderate stenosis and higher than

10mmHg for severe stenosis. This type of measurement allows for a surgeon to evaluate the

complexity of a patient’s stenosis and their need for possible mitral valve repair or mitral valve

replacement.

Diastolic and Systolic: Systole is the contraction of ventricles and diastole is the complete

relaxation of the ventricles; however, to better understand the mitral valve it is important to

understand this topic in greater depth. Murmurs usually indicate specific heart disease or

malformation.The timing of a murmur is crucial to accurate diagnosis. A murmur is either

systolic, diastolic or continuous throughout systole and diastole. Systole occurs between the S1

and S2 heart sounds, while diastole occurs between S2 and S1. A systolic murmur could mean

mitral regurgitation or mitral valve prolapse and a diastolic murmur could mean mitral valve

stenosis.

Left Atrial Diameter: The left atrial diameter is measured at end systole. Left atrial

dilation reflects long-term increased filling pressure which is found with mitral valve disease,

stenosis, and regurgitation. Mild to severe diameters range from 41mm to 52mm or higher. The

left ventricular end diastolic diameter is the most important measurement in determining cardiac

disease and the largest cardiac dimension. Moderate to severe dilation can range from 58-69 mm.
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Left Ventricular Ejection Fraction: The left ventricular ejection fraction is the

measurement of how much blood is being pumped out of the left ventricle with each contraction.

Moderate to severe ejection fractions are 35% to 39% or higher, and is calculated by dividing the

stroke volume with the end diastolic volume and multiplying that number by 100.

Mitral Annulus (better touched upon in ​Mitral Valve Anatomy)​: The mitral valve

constitutes the anatomical junction between the left ventricle and atrium, and serves as an

insertion site for leaflet tissue. Understanding a patient’s annulus can help diagnose a patient’s

specific malformation and again treatment plan.

Z- Score: The z- score is a means of expressing the deviation of a given measurement

within a population. This is highly considered in pediatric because since a child grows the

normal standard measures change with age and size. If a patient is above population mean the z-

score will be positive and if a patient is below population mean the z-score will be negative.

Conducting Medical Research

Medical research is a fairly general topic and can vary greatly depending on each

research. However writing a publication follows a similar process. The first step into writing a

medical research paper is to prioritize and schedule time for writing through writing

appointments, which build practice and confidence. Like any paper a research publication starts

with an outline. The first level of an outline should answer the following questions: What is the

topic of my paper? Why is this topic important? How could I formulate my hypothesis? What are

my results? What is my major finding? The next level adds more structure to the outline by

grouping ideas into sections: Introduction, Methods, Results, Discussion, and Conclusion. The

most important step into continuing this outline is communication within the research team and
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an overall collaboration of ideas. The next step is to create a draft of the official publication. This

includes expanding on each idea within the outline, adding data, and elaborating on detail. The

improvement of medicine comes from the continuous share of ideas within curious scientists. It

is important to avoid over editing, but instead focus on pouring every idea learned through the

research.

Work Cited

Seguela, Pierre- Emmanuel, Lucile Houyel, and Phiilippe Acar. "Congenital Malformations of

the Mitral Valve." ​Archives of Cardiovascular Disease​ : 465-79. ​Elsevier Masson​. Web.

Ohno, Hideaki, Yasuharu Imai, Masatsugu Terada, and Takeshi Hiramatsu. "The Long-Term

Results of Commissure Plication Annuloplasty for Congenital Mitral Insufficiency."

Annals of Thoracic Surgery​ 68 (1999): 537-41. Web.

Shi, Yi, Haitao Xu, Qiang Wang, Shoujun Li, Tong Yi, Yajuan Zhang, and Wenchao Liu. "The

Mid-term Results of Mitral Valve Repair for Isolated Mitral Regurgitation in Infancy and

Childhood." ​Pediatric Cardiology​ (2017): n. pag. ​Springer Science+Business Media​.

Web.

Chubb, Henry, and John M Simpson. “The Use of Z-Scores in Paediatric Cardiology.” ​Annals of

Pediatric Cardiology​ 5.2 (2012): 179–184. ​PMC​. Web. 30 Nov. 2017.

Collier, Patrick, et al. “Mitral Valve Disease: Stenosis and Regurgitation.” ​Mitral Valve Disease:

Stenosis and Regurgitation​, Aug. 2014. ​Cleveland Clinic​,

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www.clevelandclinicmeded.com/medicalpubs/diseasemanagement/cardiology/mitral-val

ve-disease/.

“Echocardiography in ICU.” ​Left Atrium Dimensions - Echocardiography in ICU​,

web.stanford.edu/group/ccm_echocardio/cgi-bin/mediawiki/index.php/Left_atrium_dim

ensions.

“Ejection Fraction.” ​Cleveland Clinic​, my.clevelandclinic.org/health/articles/ejection-fraction.

Gross, Dr. Kenneth. ​E-Echocardiography​,

e-echocardiography.com/page/page.php?UID=1867001.

“Left Ventricle Size- Echocardiography in ICU.” ​Echocardiography in the ICU​, Stanford,

web.stanford.edu/group/ccm_echocardio/cgi-bin/mediawiki/index.php/Left_ventricle_s

ize.

“Mitral Annulus.” ​Mitral Annulus : Mitral Valve Repair Reference Center​,

www.mitralvalverepair.org/content/view/54/.

“Mitral Stenosis.” ​Mitral Stenosis - Wikiecho​, 4 Sept. 2013,

www.wikiecho.org/wiki/Mitral_Stenosis#Mean_transmitral_valve_gradient.

“Mitral Stenosis.” ​Mitral Stenosis - Wikiecho​, 4 Sept. 2013,

www.wikiecho.org/wiki/Mitral_Stenosis#Mean_transmitral_valve_gradient.

“Systolic and Diastolic Murmurs.” ​Image for Cardiovascular Physiology Concepts, Richard E

Klabunde PhD​, ​www.cvphysiology.com/Heart%20Disease/HD008​.

Kallestinova, Elena D. “How to Write Your First Research Paper.” ​The Yale Journal of Biology

and Medicine​ 84.3 (2011): 181–190. Print.

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Süt, Necdet. “Study Designs in Medicine.” ​Balkan Medical Journal​ 31.4 (2014): 273–277.

PMC​. Web. 8 Dec. 2017.

“ Clinical Trials Cardiology Facts and Pearls Cardiology Mnemonics Cardiology Guidelines

Patient Information Describing Murmurs.” Healio.com,

www.healio.com/cardiology/learn-the-heart/cardiology-review/topic-reviews/describing

-murmurs.