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

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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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