Ventricular septal defect

Last updated
Ventricular septal defect
Vsd simple-lg.jpg
Illustration showing various forms of ventricular septal defects.
1. Conoventricular, malaligned
2. Perimembranous
3. Inlet
4. Muscular
Specialty Cardiac surgery
Heart sounds of a ventricular septal defect in a 14-year-old girl.

A ventricular septal defect (VSD) is a defect in the ventricular septum, the wall dividing the left and right ventricles of the heart. The extent of the opening may vary from pin size to complete absence of the ventricular septum, creating one common ventricle. The ventricular septum consists of an inferior muscular and superior membranous portion and is extensively innervated with conducting cardiomyocytes.

Contents

The membranous portion, which is close to the atrioventricular node, is most commonly affected in adults and older children in the United States. [1] It is also the type that will most commonly require surgical intervention, comprising over 80% of cases. [2]

Membranous ventricular septal defects are more common than muscular ventricular septal defects, and are the most common congenital cardiac anomaly. [3]

Signs and symptoms

Ventricular septal defect is usually symptomless at birth. It usually manifests a few weeks after birth.[ citation needed ]

VSD is an acyanotic congenital heart defect, aka a left-to-right shunt, so there are no signs of cyanosis in the early stage. However, uncorrected VSD can increase pulmonary resistance leading to the reversal of the shunt and corresponding cyanosis.[ citation needed ]

The restrictive ventricular septal defects (smaller defects) are associated with a louder murmur and more palpable thrill (grade IV murmur). Larger defects may eventually be associated with pulmonary hypertension due to the increased blood flow. Over time this may lead to an Eisenmenger's syndrome the original VSD operating with a left-to-right shunt, now becomes a right-to-left shunt because of the increased pressures in the pulmonary vascular bed.

Cause

Congenital VSDs are frequently associated with other congenital conditions, such as Down syndrome. [5] Congenital heart disease, particularly VSDs, is the number one cause of death for children with Down syndrome ages birth to two. [6]

A VSD can also form a few days after a myocardial infarction [7] (heart attack) due to mechanical tearing of the septal wall, before scar tissue forms, when macrophages start remodeling the dead heart tissue.

A congenital VSD can result from a disturbance in the morphogenesis of the heart in its embryonic stages. In the fifth week of gestation, the heart undergoes multiple processes of septation and forming a dextral loop. Interfering with the latter leads to insufficient leftward movement of the ventricular outflow tract over the atrioventricular canal, which in turn can result in a VSD or, in the most extreme cases, a double outlet right ventricle with one. [8] [9]

A ventricular septal defect arises when the superior part of the interventricular septum, which separates the right and left ventricles of the heart, fails to fully develop. The right ventricle pumps blood to the lungs to get oxygen, while the left ventricle pumps blood to the rest of the body to provide oxygen to tissues. A ventricular septal defect results in the mixing of oxygen-rich blood with oxygen-poor blood, increasing strain on the heart and lungs. [10]

Pathophysiology

During ventricular contraction, or systole, some of the blood from the left ventricle leaks into the right ventricle, passes through the lungs and reenters the left ventricle via the pulmonary veins and left atrium. This has two net effects. First, the circuitous refluxing of blood causes volume overload on the left ventricle. Second, because the left ventricle normally has a much higher systolic pressure (~120 mmHg) than the right ventricle (~20 mmHg), the leakage of blood into the right ventricle therefore elevates right ventricular pressure and volume, causing pulmonary hypertension with its associated symptoms.

In serious cases, the pulmonary arterial pressure can reach levels that equal the systemic pressure. This reverses the left to right shunt, so that blood then flows from the right ventricle into the left ventricle, resulting in cyanosis, as blood is by-passing the lungs for oxygenation. [11]

This effect is more noticeable in patients with larger defects, who may present with breathlessness, poor feeding and failure to thrive in infancy. Patients with smaller defects may be asymptomatic. Four different septal defects exist, with perimembranous most common, outlet, atrioventricular, and muscular less commonly. [12]

Diagnosis

Echocardiographic image of a moderate ventricular septal defect in the mid-muscular part of the septum. The trace in the lower left shows the flow during one complete cardiac cycle and the red mark the time in the cardiac cycle that the image was captured. Colours are used to represent the velocity of the blood. Flow is from the left ventricle (right on image) to the right ventricle (left on image). The size and position is typical for a VSD in the newborn period. Ventricular Septal Defect.jpg
Echocardiographic image of a moderate ventricular septal defect in the mid-muscular part of the septum. The trace in the lower left shows the flow during one complete cardiac cycle and the red mark the time in the cardiac cycle that the image was captured. Colours are used to represent the velocity of the blood. Flow is from the left ventricle (right on image) to the right ventricle (left on image). The size and position is typical for a VSD in the newborn period.

A VSD can be detected by cardiac auscultation. Classically, a VSD causes a pathognomonic holo- or pansystolic murmur. Auscultation is generally considered sufficient for detecting a significant VSD. The murmur depends on the abnormal flow of blood from the left ventricle, through the VSD, to the right ventricle. If there is not much difference in pressure between the left and right ventricles, then the flow of blood through the VSD will not be very great and the VSD may be silent. This situation occurs a) in the fetus (when the right and left ventricular pressures are essentially equal), b) for a short time after birth (before the right ventricular pressure has decreased), and c) as a late complication of unrepaired VSD. Confirmation of cardiac auscultation can be obtained by non-invasive cardiac ultrasound (echocardiography). To more accurately measure ventricular pressures, cardiac catheterization, can be performed.

Classification

Although there are several classifications for VSD, the most accepted and unified classification is that of Congenital Heart Surgery Nomenclature and Database Project. [13] The classification is based on the location of the VSD on the right ventricular surface of the inter ventricular septum and is as follows:

Multiple

Type 1

Type 1 is sub aortic

Type 2

  • Type 2 also known as perimembranous, paramembranous, conoventricular, membranous septal defect, and subaortic.
  • Most common variety found in 70%

Type 3

Type 3 also known as inlet (or AV canal type).

Type 4

Type 4 also known as muscular (trabecular)

  • Located in the muscular septum, found in 20%. Can be sub classified again based on the location into anterior, apical, posterior and mid

Type: Gerbode

Type: Gerbode also known as left ventricular to right atrial communication

  • Due to absence of Atrioventricular septum.

Treatment

A nitinol device for closing muscular VSDs, 4 mm diameter in the centre. It is shown mounted on the catheter into which it will be withdrawn during insertion. VSD plug.jpg
A nitinol device for closing muscular VSDs, 4 mm diameter in the centre. It is shown mounted on the catheter into which it will be withdrawn during insertion.

Most cases do not need treatment and heal during the first years of life. Treatment is either conservative or surgical. Smaller congenital VSDs often close on their own, as the heart grows, and in such cases may be treated conservatively. Some cases may necessitate surgical intervention, i.e. with the following indications:

  1. Failure of congestive cardiac failure to respond to medications
  2. VSD with pulmonic stenosis
  3. Large VSD with pulmonary hypertension
  4. VSD with aortic regurgitation

For the surgical procedure, a heart-lung machine is required and a median sternotomy is performed. Percutaneous endovascular procedures are less invasive and can be done on a beating heart, but are only suitable for certain patients. Repair of most VSDs is complicated by the fact that the conducting system of the heart is in the immediate vicinity.

Ventricular septum defect in infants is initially treated medically with cardiac glycosides (e.g., digoxin 10-20 μg/kg per day), loop diuretics (e.g., furosemide 1–3 mg/kg per day) and ACE inhibitors (e.g., captopril 0.5–2 mg/kg per day).

Transcatheter closure

A device, known as the Amplatzer muscular VSD occluder, may be used to close certain VSDs. [14] It was initially approved in 2009. [14] It appears to work well and be safe. [14] The cost is also lower than having open heart surgery. [14] The device is placed through a small incision in the groin. [15]

The Amplatzer septal occluder was shown to have full closure of the ventricular defect within the 24 hours of placement. [16] It has a low risk of embolism after implantation. [17] Some tricuspid valve regurgitation was shown after the procedure that could possibly be from the right ventricular disc. [16] There have been some reports that the Amplatzer septal occluder may cause life-threatening erosion of the tissue inside the heart. [18] This occurs in one percent of people implanted with the device and requires immediate open-heart surgery. [18] This erosion occurs due to improper sizing of the device resulting with it being too large for the defect, causing rubbing of the septal tissue and erosion. [18]

Surgery

Surgical closure of a Perimembranous VSD is performed on cardiopulmonary bypass with ischemic arrest. Patients are usually cooled to 28 degrees. Percutaneous Device closure of these defects is rarely performed in the United States because of the reported incidence of both early and late onset complete heart block after device closure, presumably secondary to device trauma to the AV node.

Surgical exposure is achieved through the right atrium. The tricuspid valve septal leaflet is retracted or incised to expose the defect margins.

Several patch materials are available, including native pericardium, bovine pericardium, PTFE (Gore-Tex or Impra), or Dacron.

Suture techniques include horizontal pledgeted mattress sutures, and running polypropylene suture.

Critical attention is necessary to avoid injury to the conduction system located on the left ventricular side of the interventricular septum near the papillary muscle of the conus. Care is taken to avoid injury to the aortic valve with sutures.

Once the repair is complete, the heart is extensively deaired by venting blood through the aortic cardioplegia site, and by infusing Carbon Dioxide into the operative field to displace air.

Intraoperative transesophageal echocardiography is used to confirm secure closure of the VSD, normal function of the aortic and tricuspid valves, good ventricular function, and the elimination of all air from the left side of the heart.

The sternum, fascia and skin are closed, with potential placement of a local anesthetic infusion catheter under the fascia, to enhance postoperative pain control.

Multiple muscular VSDs are a challenge to close, achieving a complete closure can be aided by the use of fluorescein dye. [19]

Epidemiology

VSDs are the most common congenital cardiac abnormalities. They are found in 30-60% of all newborns with a congenital heart defect, or about 2-6 per 1000 births. During heart formation, when the heart begins life as a hollow tube, it begins to partition, forming septa. If this does not occur properly it can lead to an opening being left within the ventricular septum. It is debatable whether all those defects are true heart defects, or if some of them are normal phenomena, since most of the trabecular VSDs close spontaneously. [20] Prospective studies give a prevalence of 2-5 per 100 births of trabecular VSDs that close shortly after birth in 80-90% of the cases. [21] [22]

Famous people who had ventricular septal defect

See also

Related Research Articles

<span class="mw-page-title-main">Heart valve</span> A flap of tissue that prevent backflow of blood around the heart

A heart valve is a biological one-way valve that allows blood to flow in one direction through the chambers of the heart. Four valves are usually present in a mammalian heart and together they determine the pathway of blood flow through the heart. A heart valve opens or closes according to differential blood pressure on each side.

<span class="mw-page-title-main">Heart sounds</span> Noise generated by the beating heart

Heart sounds are the noises generated by the beating heart and the resultant flow of blood through it. Specifically, the sounds reflect the turbulence created when the heart valves snap shut. In cardiac auscultation, an examiner may use a stethoscope to listen for these unique and distinct sounds that provide important auditory data regarding the condition of the heart.

<span class="mw-page-title-main">Heart murmur</span> Medical condition

Heart murmurs are unique heart sounds produced when blood flows across a heart valve or blood vessel. This occurs when turbulent blood flow creates a sound loud enough to hear with a stethoscope. The sound differs from normal heart sounds by their characteristics. For example, heart murmurs may have a distinct pitch, duration and timing. The major way health care providers examine the heart on physical exam is heart auscultation; another clinical technique is palpation, which can detect by touch when such turbulence causes the vibrations called cardiac thrill. A murmur is a sign found during the cardiac exam. Murmurs are of various types and are important in the detection of cardiac and valvular pathologies.

<span class="mw-page-title-main">Tetralogy of Fallot</span> Type of congenital heart defect

Tetralogy of Fallot (TOF), formerly known as Steno-Fallot tetralogy, is a congenital heart defect characterized by four specific cardiac defects. Classically, the four defects are:

<span class="mw-page-title-main">Ventricle (heart)</span> Chamber of the heart

A ventricle is one of two large chambers located toward the bottom of the heart that collect and expel blood towards the peripheral beds within the body and lungs. The blood pumped by a ventricle is supplied by an atrium, an adjacent chamber in the upper heart that is smaller than a ventricle. Interventricular means between the ventricles, while intraventricular means within one ventricle.

dextro-Transposition of the great arteries Medical condition

dextro-Transposition of the great arteries is a potentially life-threatening birth defect in the large arteries of the heart. The primary arteries are transposed.

<span class="mw-page-title-main">Atrial septal defect</span> Human heart defect present at birth

Atrial septal defect (ASD) is a congenital heart defect in which blood flows between the atria of the heart. Some flow is a normal condition both pre-birth and immediately post-birth via the foramen ovale; however, when this does not naturally close after birth it is referred to as a patent (open) foramen ovale (PFO). It is common in patients with a congenital atrial septal aneurysm (ASA).

<span class="mw-page-title-main">Congenital heart defect</span> Defect in the structure of the heart that is present at birth

A congenital heart defect (CHD), also known as a congenital heart anomaly, congenital cardiovascular malformation, and congenital heart disease, is a defect in the structure of the heart or great vessels that is present at birth. A congenital heart defect is classed as a cardiovascular disease. Signs and symptoms depend on the specific type of defect. Symptoms can vary from none to life-threatening. When present, symptoms are variable and may include rapid breathing, bluish skin (cyanosis), poor weight gain, and feeling tired. CHD does not cause chest pain. Most congenital heart defects are not associated with other diseases. A complication of CHD is heart failure.

The Rastelli procedure is an open heart surgical procedure developed by Italian physician and cardiac surgery researcher, Giancarlo Rastelli, in 1967 at the Mayo Clinic, and involves using a pulmonary or aortic homograft conduit to relieve pulmonary obstruction in double outlet right ventricle with pulmonary stenosis.

<span class="mw-page-title-main">Pulmonary atresia</span> Medical condition

Pulmonary atresia is a congenital malformation of the pulmonary valve in which the valve orifice fails to develop. The valve is completely closed thereby obstructing the outflow of blood from the heart to the lungs. The pulmonary valve is located on the right side of the heart between the right ventricle and pulmonary artery. In a normal functioning heart, the opening to the pulmonary valve has three flaps that open and close.

<span class="mw-page-title-main">Tricuspid atresia</span> Medical condition

Tricuspid atresia is a form of congenital heart disease whereby there is a complete absence of the tricuspid valve. Therefore, there is an absence of right atrioventricular connection. This leads to a hypoplastic (undersized) or absent right ventricle. This defect is contracted during prenatal development, when the heart does not finish developing. It causes the systemic circulation to be filled with relatively deoxygenated blood. The causes of tricuspid atresia are unknown.

<span class="mw-page-title-main">Atrioventricular septal defect</span> Medical condition

Atrioventricular septal defect (AVSD) or atrioventricular canal defect (AVCD), also known as "common atrioventricular canal" or "endocardial cushion defect" (ECD), is characterized by a deficiency of the atrioventricular septum of the heart that creates connections between all four of its chambers. It is a very specific combination of 3 defects:

<span class="mw-page-title-main">Interventricular septum</span> Wall of tissue separating ventricles of human heart

The interventricular septum is the stout wall separating the ventricles, the lower chambers of the heart, from one another.

Levo-Transposition of the great arteries is an acyanotic congenital heart defect in which the primary arteries are transposed, with the aorta anterior and to the left of the pulmonary artery; the morphological left and right ventricles with their corresponding atrioventricular valves are also transposed.

A right-to-left shunt is a cardiac shunt which allows blood to flow from the right heart to the left heart. This terminology is used both for the abnormal state in humans and for normal physiological shunts in reptiles.

<span class="mw-page-title-main">Foramen secundum</span>

The foramen secundum or ostium secundum is a foramen in the septum primum, a precursor to the interatrial septum of the human heart.

A cardiac shunt is a pattern of blood flow in the heart that deviates from the normal circuit of the circulatory system. It may be described as right-left, left-right or bidirectional, or as systemic-to-pulmonary or pulmonary-to-systemic. The direction may be controlled by left and/or right heart pressure, a biological or artificial heart valve or both. The presence of a shunt may also affect left and/or right heart pressure either beneficially or detrimentally.

<span class="mw-page-title-main">Lutembacher's syndrome</span> Medical condition

Lutembacher's syndrome is a very rare form of congenital heart disease that affects one of the chambers of the heart as well as a valve. It is commonly known as both congenital atrial septal defect (ASD) and acquired mitral stenosis (MS). Congenital atrial septal defect refers to a hole being in the septum or wall that separates the two atria; this condition is usually seen in fetuses and infants. Mitral stenosis refers to mitral valve leaflets sticking to each other making the opening for blood to pass from the atrium to the ventricles very small. With the valve being so small, blood has difficulty passing from the left atrium into the left ventricle. Septal defects that may occur with Lutembacher's syndrome include: Ostium primum atrial septal defect or ostium secundum which is more prevalent.

<span class="mw-page-title-main">Ventricular outflow tract obstruction</span> Medical condition

A ventricular outflow tract obstruction is a heart condition in which either the right or left ventricular outflow tract is blocked or obstructed. These obstructions represent a spectrum of disorders. Majority of these cases are congenital, but some are acquired throughout life.

References

  1. Taylor, Michael D (2019-02-02). "Muscular Ventricular Septal Defect". EMedicine. Medscape.
  2. Waight, David J.; Bacha, Emile A.; Kahana, Madelyn; Cao, Qi-Ling; Heitschmidt, Mary; Hijazi, Ziyad M. (March 2002). "Catheter therapy of Swiss cheese ventricular septal defects using the Amplatzer muscular VSD occluder". Catheterization and Cardiovascular Interventions. 55 (3): 355–361. doi:10.1002/ccd.10124. PMID   11870941. S2CID   23602868.
  3. Hoffman, JI; Kaplan, S (2002). "The incidence of congenital heart disease". Journal of the American College of Cardiology. 39 (12): 1890–900. doi: 10.1016/S0735-1097(02)01886-7 . PMID   12084585.
  4. Cameron P. et al: Textbook of Pediatric Emergency Medicine. p116-117 [Elsevier, 2006]
  5. Wells, GL; Barker, SE; Finley, SC; Colvin, EV; Finley, WH (1994). "Congenital heart disease in infants with Down's syndrome". Southern Medical Journal. 87 (7): 724–7. doi:10.1097/00007611-199407000-00010. PMID   8023205. S2CID   31622875.
  6. Benhaourech, S; Dirgil, A; Hammiri, AE (2016). "Congenital heart disease and Down syndrome: various aspects of a confirmed association". Cardiovasc J Afr. 27 (5): 287–290. doi:10.5830/CVJA-2016-019. PMC   5370349 . PMID   27805241.
  7. Schumacher, Kurt R. "Ventricular septal defect". NIH and US National Library of Medicine. MedlinePlus.
  8. Lamers, Wouter H.; Moorman, Antoon F.M. (2002-07-26). "Cardiac Septation – A Late Contribution of the Embryonic Primary Myocardium to Heart Morphogenesis". Circulation Research . 91 (2): 93–103. doi: 10.1161/01.RES.0000027135.63141.89 . PMID   12142341.
  9. Gittenberger-de Groot, Adriana C.; Bartelings, Margot M.; Deruiter, Marco C.; Poelmann, Robert E. (2005-02-01). "Basics of Cardiac Development for the Understanding of Congenital Heart Malformations". Pediatric Research . 57 (2): 170. doi: 10.1203/01.PDR.0000148710.69159.61 . PMID   15611355.
  10. "Congenital Heart Defects - What are Congenital Heart Defects? | NHLBI, NIH". 24 March 2022.
  11. Kumar & Clark 2009
  12. Mancini, Mary C (2018-06-20). "Ventricular Septal Defect Surgery in the Pediatric Patient". EMedicine. Medscape.
  13. Jacobs, Jeffrey; Mavroudis, Constantine (March 2000). "Congenital Heart Surgery Nomenclature and Database Project: ventricular septal defect". Ann Thorac Surg. 69 (3): 25–35. doi:10.1016/S0003-4975(99)01270-9. PMID   10798413.
  14. 1 2 3 4 Fu, YC (February 2011). "Transcatheter device closure of muscular ventricular septal defect". Pediatrics and Neonatology. 52 (1): 3–4. doi: 10.1016/j.pedneo.2010.12.012 . PMID   21385649.
  15. Amplatzer septal occluder. (2013) U.S. Food and Drug Administration. Retrieved February 26, 2014, from https://www.fda.gov/MedicalDevices/ProductsandMedicalProcedures/DeviceApprovalsandClearances/Recently-ApprovedDevices/ucm083978.htm
  16. 1 2 Szkutnik; et al. (2007). "Use of the Amplatzer muscular ventricular septal defect occluder for closure of perimembranous ventricular septal defects". Heart. 93 (3): 355–358. doi:10.1136/hrt.2006.096321. PMC   1861424 . PMID   16980519.
  17. Fernando Rajeev; et al. (2013). "Patent ductus arteriosus closure using an Amplatzer ventricular septal defect closure device". Experimental & Clinical Cardiology . 18 (1): e50–e54.
  18. 1 2 3 Rare Serious Erosion Events Associated with St. Jude Amplatzer Atrial Septal Occluder (ASO). (2013, October 17). U.S. Food and Drug Administration. Retrieved February 26, 2014, from https://www.fda.gov/MedicalDevices/Safety/AlertsandNotices/ucm371145.htm
  19. Mathew, Thomas (2014). "Use of Fluorescein Dye to Identify Residual Defects". Ann Thorac Surg. 97 (1): e27-8. doi:10.1016/j.athoracsur.2013.10.059. ISSN   0003-4975. PMID   24384220.
  20. Meberg, A; Otterstad, JE; Frøland, G; Søarland, S; Nitter-Hauge, S (1994). "Increasing incidence of ventricular septal defects caused by improved detection rate". Acta Paediatrica. 83 (6): 653–657. doi:10.1111/j.1651-2227.1994.tb13102.x. PMID   7919765. S2CID   30244380.
  21. Hiraishi, S; Agata, Y; Nowatari, M; Oguchi, K; Misawa, H; Hirota, H; Fujino, N; Horiguchi, Y; Yashiro, K; Nakae, S (March 1992). "Incidence and natural course of trabecular ventricular septal defect: two-dimensional echocardiography and color Doppler flow imaging study". The Journal of Pediatrics. 120 (3): 409–15. doi:10.1016/s0022-3476(05)80906-0. PMID   1538287.
  22. Roguin, Nathan; Du, Zhong-Dong; Barak, Mila; Nasser, Nadim; Hershkowitz, Sylvia; Milgram, Elliot (15 November 1995). "High prevalence of muscular ventricular septal defect in neonates". Journal of the American College of Cardiology. 26 (6): 1545–1548. doi: 10.1016/0735-1097(95)00358-4 . PMID   7594083.
  23. "The blue baby syndrome". Deccan Herald . 25 September 2015. Retrieved 15 July 2022.
  24. Reiss, Jonathan (June 9, 2020). Look at Me!. Hachette Books. p. 1. ISBN   978-0-306-84541-3 . Retrieved 28 October 2022.