Crisscross heart

Crisscross heart
Other namesCriss-cross atrioventricular relationships[1]
SpecialtyCardiology

Crisscross heart is a type of congenital heart defect where the right atrium is closely associated with the left ventricle in space, and the left atrium is closely associated with the right ventricle.[2] The first documented report of this heart defect was described in 1961 by Dr. Robert H. Anderson, Dr. Elliot A. Shinebourne, and Dr. Leon M. Gerlis[3].

Although it is classified as a defect, the criss-cross is more of a spatial anomaly than a functional one, and it is possible for the heart to have relatively normal functioning. The ventricles are rotated either clockwise or counterclockwise resulting in the twisting of their connection.[4] The actual blood flow stream through the ventricles is not interrupted.

Etiology

The exact cause of the heart defect is unknown, but it is postulated to be early embryological rotation of the ventricular mass along the long-axis[5] after the ventricular septum forms around the 7th week of embryologic development.

Symptoms and signs

Depending on when the crossing of the ventricles occurs during fetal development dictates the severity of symptoms and anatomical defects the individual will experience. Early rotation of the ventricular mass before the septum is fully formed can cause varying degrees of ventricular septal defects to persist leading to shortness of breath, cyanosis, pulmonary arterial hypertension, and possible formation of a single ventricle.[5]

Crisscross heart is a very rare congenital heart defect, and results in many different symptoms, even though the heart still has the ability to perform its major function of pumping blood throughout the body. Individuals who have this disease will experience cyanosis which is a blue tint to the skin because of inadequate blood flow to the body, this symptom will be seen especially around the mouth. Other symptoms include pallor, extreme dyspnea, pulmonary valve stenosis, cardiac murmurs[6] and a deviated ventricular septum.[7] Pallor can be described as a pale color of the skin, and dyspnea is difficulty breathing. Pulmonary valve stenosis is the narrowing of the pulmonary valve which leads to decreased blood flow to the pulmonary artery. Cardiac murmurs are sounds that can be heard when using a stethoscope that make a swooshing noise rather than a normal “lub-dup”. Lastly a deviated ventricular septum is when there is a hole between the ventricle walls resulting in blood between the ventricles flowing freely between each other.[8]

Anatomy

In an anatomically correct heart the right atrium and right ventricle are working together to supply blood to the pulmonary artery, similarly to how the left atrium and the left ventricle work simultaneously to supply blood to the aorta. During the process of the heart contracting and releasing the right atrium and left atrium contract at the same time, while the left ventricle and right ventricle relax. In opposition, when the left atrium and right atrium are relaxed the left ventricle and right ventricle contract pushing blood to either the aorta or pulmonary artery. In an anatomically correct heart the atria are smaller than the ventricles. The ventricles include more muscle in order to push high quantities of blood throughout the body. Normal blood flow throughout the heart begins at the superior vena cava coming from the upper half of the body and the inferior vena cava coming from the lower half of the body. Next blood will be in the right atrium and will flow uninterrupted through the tricuspid valve through to the right ventricle. The blood from the right ventricle should go to the pulmonary artery via the pulmonary valve. The blood from the pulmonary vein enters the left atrium, then flows through the mitral valve to the left ventricle. After the left ventricle is filled with blood the aortic valve opens allowing blood to go through, which the blood then enters the aorta and goes to the rest of the body.[9]

Diagnosis

Crisscross heart can be diagnosed by an echocardiogram, angiocardiogram, and a cardiac MRI. To diagnose crisscross heart during embryonic development a sonogram will be used, and it is very important identify the disease prenatally so the child can be treated immediately for better cardiac function in adulthood.[10]

Treatment

The rotated ventricles indicative of crisscross heart generally pose no threat to the patient. But associated severe anatomic defects can be fatal if left unaddressed. Definitive treatment is aimed at fixing large septal defects and pulmonary valve stenosis rather than the rotated heart itself. Due to the complexity of the native heart anatomy, staged surgical repair with careful planning through various imaging modalities is necessary.

References

  1. ^ RESERVED, INSERM US14-- ALL RIGHTS. "Orphanet: Criss cross heart". www.orpha.net. Retrieved 27 May 2019.{{cite web}}: CS1 maint: numeric names: authors list (link)
  2. ^ Fontes VF, de Souza JA, Pontes Jùnior SC (March 1990). "Criss-cross heart with intact ventricular septum". International Journal of Cardiology. 26 (3): 382–5. doi:10.1016/0167-5273(90)90102-B. PMID 2312210.
  3. ^ Anderson, Robert H.; Shinebourne, Elliot A.; Gerlis, Leon M. (1974-07). "Criss-Cross Atrioventricular Relationships Producing Paradoxical Atrioventricular Concordance or Discordance: Their Significance to Nomenclature of Congenital Heart Disease". Circulation. 50 (1): 176–180. doi:10.1161/01.CIR.50.1.176. ISSN 0009-7322. {{cite journal}}: Check date values in: |date= (help)
  4. ^ Angelini P (2010). "Left ventricle on top versus right ventricle on top in superoinferior ventricles: what are we talking about?". Texas Heart Institute Journal. 37 (4): 442–4. PMC 2929877. PMID 20844618.
  5. ^ a b Kadermuneer, P.; Thottian, Julian Johny; Rajesh, Kadukanmackil Francis; Sajeev, Chakanalil Govindan; Krishnan, Mangalath Narayanan (2015-07). "'A twist in the heart' – Echocardiographic diagnosis of criss-cross heart". Journal of Cardiology Cases. 12 (1): 23–25. doi:10.1016/j.jccase.2015.04.004. {{cite journal}}: Check date values in: |date= (help)
  6. ^ "Gale - Product Login". go.galegroup.com. Retrieved 2018-10-25.
  7. ^ RESERVED, INSERM US14 -- ALL RIGHTS. "Orphanet: Criss cross heart". www.orpha.net. Retrieved 2018-10-25.{{cite web}}: CS1 maint: numeric names: authors list (link)
  8. ^ Taksande AM (April 2013). "Echocardiographic recognition of a criss-cross heart with double outlet right ventricle". Images in Paediatric Cardiology. 15 (2): 3–7. PMC 3669543. PMID 23847656.
  9. ^ Philadelphia, The Children's Hospital of (2014-08-10). "How the Normal Heart Works". www.chop.edu. Retrieved 2018-10-25.
  10. ^ Li S, Luo G, Norwitz ER, Wang C, Ouyang S, Yao Y, Wen H, Chen C, Fu Q, Xia X, Bi J, Zhu J (February 2013). "Prenatal diagnosis of criss-cross heart: sonographical and pathological features of five cases". Journal of Perinatology. 33 (2): 98–102. doi:10.1038/jp.2012.56. PMID 22555779.

Further reading

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