Isolated levocardia

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Isolated levocardia (also known as situs inversus with levocardia) is a rare type of organs' situs inversus in which the heart is still in normal position but other abdominal viscera are transposed. [1] [2] Isolated levocardia may occur with heart defects and patients without having operations have low life expectancy: only about 5% to 13% of patients survive more than 5 years. [3] Therefore, even though the risk of cardiac surgeries is high, once patients are diagnosed, operations are suggested to be held as soon as possible. Isolated levocardia is congenital. So far, there is not sufficient evidence to prove that chromosome abnormalities will result in isolated levocardia, and the cause of isolated levocardia is still unknown.

Contents

Symptoms

Symptoms are dependent upon complications. Isolated levocardia without complications may have no symptoms. Symptoms of untreated cardiac defects may range from low exercise tolerance to death.

Diagnosis

Systematic examination is need to be diagnosed. To determine the position of organs and major blood vessels of isolated levocardia patients, ultrasonography, CT, and MRI are used alone and in combination: [4]

Through data comparison, the average value of cardiovascular malformation in levocardia patients is 4.8±1.8, and for isolated levocardia, the average value is 3.0±1.7. [6]

Through the analysis of isolated levocardia cases, some common features of isolated levocardia patients are:

Incidence & complications

In general population, the incidence of isolated levocardia is 1: 22,000; in all patients with congenital heart disease, the incidence is from 0.4% to 1.2%.

There are extensive cases reporting that children and adults with isolated levocardia have complex cardiac defects, [7] and neonate or infant with isolated levocardia may be complicated by bowel obstruction and cardiac anomalies. [8] It is estimated that up to 95% of cases of isolated levocardia have associated cardiac deformities like right ventricular outflow tract (RVOT) obstruction, septal defects, inversion of cardiac chambers and transposition of cardiac chambers. [9]

Prevention & treatment

Examination

Isolated levocardia is congenital, and so far, there is no efficient way to prevent it. However, in the prenatal period, a prenatal evaluation is suggested. It is recommended to include determination of the situs, identification of heart anomalies, and detection of the major vessels and abdominal viscera in the prenatal evaluation. In the postnatal period, a detection of IVC interruption may be helpful and a prophylactic lifelong antibiotic should be prescribed for the neonate to treat the increase of incidence of overwhelming sepsis in congenital asplenia. Besides, parents should be counseled appropriately.

With accurate, thorough sonographic examination and if there is no congenital heart malformations and chromosomal aberrations, the risk of morbidity and mortality will decrease.

Operation

If defects of the heart or intestines are present, surgical treatment may be required. Potential surgeries include: Blalock-Taussig, total cavopulmonary connection (Fontan procedure), Rastelli procedure. Blalock-Taussig is a traditional procedure, and Fontan procedure is widely operated recently. The goal of most isolated levocardia surgeries is to rebuild parts of the heart and "redirect" the way blood flows in order to augment pulmonary blood flow.

Cardiac surgeries have high risk. Despite the advances in the operative management, morbidity and mortality remain high. Most death after receiving operations may attribute to a combination of cardiac failure and renal insufficiency. The surgical strategy chosen – hybrid versus traditional procedures, choices regarding the most appropriate pulmonary blood flow – will affect management throughout a patient's life. However, further studies are still required to identify determinants of the optimal surgical strategy in individual patients. [10]

In cases of intestinal malrotation, treatment may require a Ladd's procedure.

Some cases and operation results

Related Research Articles

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<span class="mw-page-title-main">Dextrocardia</span> Atypical heart placement on the right side of the body

Dextrocardia is a rare congenital condition in which the apex of the heart is located on the right side of the body, rather than the more typical placement towards the left. There are two main types of dextrocardia: dextrocardia of embryonic arrest and dextrocardia situs inversus. Dextrocardia situs inversus is further divided.

<i>Situs inversus</i> Condition in which organs are reversed

Situs inversus is a congenital condition in which the major visceral organs are reversed or mirrored from their normal positions. The normal arrangement of internal organs is known as situs solitus. Although cardiac problems are more common, many people with situs inversus have no medical symptoms or complications resulting from the condition, and until the advent of modern medicine, it was usually undiagnosed.

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">Fontan procedure</span> Surgical procedure used in children with univentricular hearts

The Fontan procedure or Fontan–Kreutzer procedure is a palliative surgical procedure used in children with univentricular hearts. It involves diverting the venous blood from the inferior vena cava (IVC) and superior vena cava (SVC) to the pulmonary arteries. The procedure varies for differing congenital heart pathologies. For example in tricuspid atresia, the procedure can be done where the blood does not pass through the morphologic right ventricle; i.e., the systemic and pulmonary circulations are placed in series with the functional single ventricle. Whereas in hypoplastic left heart syndrome, the heart is more reliant on the more functional right ventricle to provide blood flow to the systemic circulation. The procedure was initially performed in 1968 by Francis Fontan and Eugene Baudet from Bordeaux, France, published in 1971, simultaneously described in July 1971 by Guillermo Kreutzer from Buenos Aires, Argentina, presented at the Argentinean National Cardilogy meeting of that year and finally published in 1973.

Situs ambiguus, or heterotaxy, is a rare congenital defect in which the major visceral organs are distributed abnormally within the chest and abdomen. Clinically heterotaxy spectrum generally refers to any defect of left-right asymmetry and arrangement of the visceral organs; however, classical heterotaxy requires multiple organs to be affected. This does not include the congenital defect situs inversus, which results when arrangement of all the organs in the abdomen and chest are mirrored, so the positions are opposite the normal placement. Situs inversus is the mirror image of situs solitus, which is normal asymmetric distribution of the abdominothoracic visceral organs. Situs ambiguus can also be subdivided into left-isomerism and right isomerism based on the defects observed in the spleen, lungs and atria of the heart.

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Helen Brooke Taussig was an American cardiologist, working in Baltimore and Boston, who founded the field of pediatric cardiology. She is credited with developing the concept for a procedure that would extend the lives of children born with Tetralogy of Fallot. This concept was applied in practice as a procedure known as the Blalock-Thomas-Taussig shunt. The procedure was developed by Alfred Blalock and Vivien Thomas, who were Taussig's colleagues at the Johns Hopkins Hospital.

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<span class="mw-page-title-main">Hypoplastic left heart syndrome</span> Type of congenital heart defect

Hypoplastic left heart syndrome (HLHS) is a rare congenital heart defect in which the left side of the heart is severely underdeveloped and incapable of supporting the systemic circulation. It is estimated to account for 2-3% of all congenital heart disease. Early signs and symptoms include poor feeding, cyanosis, and diminished pulse in the extremities. The etiology is believed to be multifactorial resulting from a combination of genetic mutations and defects resulting in altered blood flow in the heart. Several structures can be affected including the left ventricle, aorta, aortic valve, or mitral valve all resulting in decreased systemic blood flow.

<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.

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The Norwood procedure is the first of three surgeries intended to create a new functional systemic circuit in patients with hypoplastic left heart syndrome (HLHS) and other complex heart defects with single ventricle physiology. The first successful Norwood procedure involving the use of a cardiopulmonary bypass was reported by Dr. William Imon Norwood, Jr. and colleagues in 1981.

Taussig–Bing syndrome is a cyanotic congenital heart defect in which the patient has both double outlet right ventricle (DORV) and subpulmonic ventricular septal defect (VSD).

<span class="mw-page-title-main">Arterial switch operation</span> Open heart surgical procedure

Arterial switch operation (ASO) or arterial switch, is an open heart surgical procedure used to correct dextro-transposition of the great arteries (d-TGA).

<span class="mw-page-title-main">Atrial septostomy</span> Surgical procedure on the heart

Atrial septostomy is a surgical procedure in which a small hole is created between the upper two chambers of the heart, the atria. This procedure is primarily used to palliate dextro-Transposition of the great arteries or d-TGA, a life-threatening cyanotic congenital heart defect seen in infants. It is performed prior to an arterial switch operation. Atrial septostomy has also seen limited use as a surgical treatment for pulmonary hypertension. The first atrial septostomy was developed by Vivien Thomas in a canine model and performed in humans by Alfred Blalock. The Rashkind balloon procedure, a common atrial septostomy technique, was developed in 1966 by American cardiologist William Rashkind at the Children's Hospital of Philadelphia.

<span class="mw-page-title-main">Bidirectional Glenn procedure</span>

The bidirectional Glenn (BDG) shunt, or bidirectional cavopulmonary anastomosis, is a surgical technique used in pediatric cardiac surgery procedure used to temporarily improve blood oxygenation for patients with a congenital cardiac defect resulting in a single functional ventricle. Creation of a bidirectional shunt reduces the amount of blood volume that the heart needs to pump at the time of surgical repair with the Fontan procedure.

<span class="mw-page-title-main">Anomalous pulmonary venous connection</span> Medical condition

Anomalous pulmonary venous connection is a congenital defect of the pulmonary veins.

<span class="mw-page-title-main">Hypoplastic right heart syndrome</span> Type of congenital heart disease

Hypoplastic right heart syndrome (HRHS) is a congenital heart defect in which the structures on the right side of the heart, particularly the right ventricle, are underdeveloped. This defect causes inadequate blood flow to the lungs, and thus a cyanotic infant.

The Senning procedure is an atrial switch heart operation performed to treat transposition of the great arteries. It is named after its inventor, the Swedish cardiac surgeon Åke Senning (1915–2000), also known for implanting the first permanent cardiac pacemaker in 1958.

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Pulmonary atresia with ventricular septal defect is a rare birth defect characterized by pulmonary valve atresia occurring alongside a defect on the right ventricular outflow tract.

References

  1. ↑ MITTAL, REKHA; PATHAK, KAMAL; MANOHARAN, S (April 1994). "ISOLATED LEVOCARDIA WITH SITUS INVERSUS (A Case Report)". Medical Journal Armed Forces India . 50 (2): 151–154. doi:10.1016/S0377-1237(17)31023-7. PMC   5529679 . PMID   28769192.
  2. ↑ Harris, Thomas R.; Rainey, Robert L. (1965-10-01). "Ideal isolated levocardia". American Heart Journal. 70 (4): 440–448. doi:10.1016/0002-8703(65)90354-6. PMID   5834189.
  3. ↑ Vijayakumar, V.; Brandt, T. (1991). "Prolonged survival with isolated levocardia and situs inversus". Cleveland Clinic Journal of Medicine. 58 (3): 243–247. doi:10.3949/ccjm.58.3.243. ISSN   0891-1150. PMID   1893555.
  4. ↑ "Isolated levocardia | Genetic and Rare Diseases Information Center (GARD) – an NCATS Program". rarediseases.info.nih.gov. Retrieved 2019-10-17.
  5. ↑ Vijayakumar, Vaniand, and Thomas Brandt (1991). "Prolonged survival with isolated levocardia and situs inversus". Cleveland Clinic Journal of Medicine. 58 (3): 243–247. doi:10.3949/ccjm.58.3.243. PMID   1893555.
  6. ↑ Hu, Jiaxin (2001). "The Diagnosis and Surgical Management of Levoversion and Isolated Levocardia". Academic Journal of Sun Yat-sen University of Medical Sciences.
  7. ↑ Gindes, L.; Hegesh, J.; Barkai, G.; Jacobson, J. M.; Achiron, R. (2007). "Isolated Levocardia: Prenatal Diagnosis, Clinical Importance, and Literature Review". Journal of Ultrasound in Medicine. 26 (3): 361–5. doi:10.7863/jum.2007.26.3.361. PMID   17324986.
  8. ↑ Gindes, Liat; Hegesh, Julian; Barkai, Gad; Jacobson, Jeffrey M.; Achiron, Reuven (2007). "Isolated Levocardia". Journal of Ultrasound in Medicine. 26 (3): 361–365. doi:10.7863/jum.2007.26.3.361. ISSN   1550-9613. PMID   17324986.
  9. ↑ Winer-Muram, Helen T. (1995). "Adult Presentation of Heterotaxic Syndromes and Related Complexes". Journal of Thoracic Imaging. 10 (1): 43–57. doi:10.1097/00005382-199501010-00004. ISSN   0883-5993. PMID   7891396. S2CID   25734073.
  10. ↑ "Hypoplastic Left Heart Syndrome Surgery: The Fontan Procedure (for Parents) - KidsHealth". kidshealth.org. Retrieved 2019-11-10.
  11. ↑ Young, Maurice D.; Griswold, Herbert E. (1951). "Situs Inversus of the Abdominal Viscera with Levocardia: Report of Eight Cases Submitted to the Blalock-Taussig Operation". Circulation. 3 (2): 202–214. doi: 10.1161/01.CIR.3.2.202 . ISSN   0009-7322. PMID   14812648.
  12. ↑ Young, Maurice D.; Griswold, Herbert E. (1951). "Situs Inversus of the Abdominal Viscera with Levocardia: Report of Eight Cases Submitted to the Blalock-Taussig Operation". Circulation. 3 (2): 202–214. doi: 10.1161/01.CIR.3.2.202 . ISSN   0009-7322. PMID   14812648.
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