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Abnormal Origin of the Pulmonary Artery

Hemitruncus Arteriosus


Presentation

  • CMR is a powerful tool in the armamentarium of pediatric cardiology and health care workers caring for patients with congenital heart disease (CHD), but a successful study still presents major technical and clinical challenges.[books.google.com]
  • Infants are usually cyanotic and may present chest pain and dyspnea on exertion.[orpha.net]
  • Presentation in adulthood is very rare.[dergipark.ulakbim.gov.tr]
  • The clinical presentation of DORV varies. On one end of the spectrum, the infant may present with profound cyanosis, while on the other end may have fulminant congestive heart failure.[news-medical.net]
  • At the present time, mortality is virtually nonexistent.[revespcardiol.org]
Coarctation of the Aorta
  • Associations It is most often an isolated anomaly, associated with other cardiac anomalies in only 5% of cases 3,7 : atrial septal defect (ASD) ventricular septal defect (VSD) patent ductus arteriosus (PDA) coarctation of the aorta tetralogy of Fallot[radiopaedia.org]
  • These other congenital heart defects are: Patent Ductus Arteriosus, Ventricular Septal Defect (VSD), Tetralogy of Fallot (TOF), and Coarctation of the Aorta (CoA).[chd-uk.co.uk]
Respiratory Distress
  • Clinical description PACA usually occurs in infant or in the newborn where it manifests by respiratory distress, recurrent respiratory infection, moderate to severe pulmonary hypertension (see this term), congestive heart failure and failure to thrive[orpha.net]
  • Respiratory distress predominates over esophageal symptoms, usually presenting early in the neonatal period. Aberrant left pulmonary arteries are thought to arise from a failure of formation of the 6 th aortic arch.[radiopaedia.org]
  • CASE REPORT A 12-day-old female baby was received with the complaints of reluctance to feed and episodes of respiratory distress since birth.[apspjcaserep.com]
  • Presenting complaints are typically nonspecific, and include diaphoresis, irritability, wheezing and respiratory distress. Precordial auscultation may reveal a murmur.[radiopaedia.org]
  • The clinical presentation includes heart failure, heart murmurs, tachypnea, dyspnea, and repeated respiratory distress.[revespcardiol.org]
Regurgitation
  • Tibayan FA, Yun KL, Fann JI, et al: Torsion dynamics in the evolution from acute to chronic mitral regurgitation. J Heart Valve Dis 2002;11:39-46.[karger.com]
  • A few weeks after the repair of the coarctation, the patient was noted to have increasing left ventricular dysfunction with increasing mitral regurgitation.[ipccc.net]
  • The LVEF of 4 patients was increased and mitral regurgitation decreased. The left ventricular performance was not worse compared to the previous condition in 3 patients.[journals.lww.com]
  • [Arunamata, 2015] Even in the setting of a normal echo, evidence of ischemia on ECG and dilated cardiomyopathy with mitral regurgitation suggests ALCAPA. [ Gonzalez, 2015 ] Cardiac CT and MRI can be useful in depicting the complex anatomy and assist with[pedemmorsels.com]
  • Physical findings include a gallop or murmur of mitral regurgitation secondary to ischemic papillary muscle dysfunction.[omicsonline.org]
Failure to Thrive
  • Clinical description PACA usually occurs in infant or in the newborn where it manifests by respiratory distress, recurrent respiratory infection, moderate to severe pulmonary hypertension (see this term), congestive heart failure and failure to thrive[orpha.net]
  • Tags: arrhythmia failure to thrive heart failure Ischemia Poor Feeding Sudden Death Sean M. Fox I enjoy taking care of patients and I finding it endlessly rewarding to help train others to do the same.[pedemmorsels.com]
Left Ventricular Dysfunction
  • Congestive heart failure (CHF) is the end result of left ventricular dysfunction in combination with significant mitral insufficiency.[radiopaedia.org]
  • Yagmur J, Sener S, Acikgoz N, et al: Subclinical left ventricular dysfunction in Behcet's disease assessed by two-dimensional speckle tracking echocardiography. Eur J Echocardiogr 2011;12:536-541.[karger.com]
  • A few weeks after the repair of the coarctation, the patient was noted to have increasing left ventricular dysfunction with increasing mitral regurgitation.[ipccc.net]
  • LV scarring and diminished LV function Leads to: Myocardial ischemia Left ventricular dysfunction Mitral insufficiency Congestive heart failure Arrhythmias Sudden Death It is a rare disease (estimates of 1 in 300,000 live births; Usually manifests as[pedemmorsels.com]
  • Consequently, the combination of left ventricular dysfunction and significant mitral valve insufficiency leads to congestive heart failure (CHF) symptoms (eg, tachypnea, poor feeding, irritability, diaphoresis) in the young infant.[chd-uk.co.uk]

Workup

  • Systemic examination and baseline laboratory workup were in normal range. X-ray chest showed bilateral perihilar infiltrates. Cardiac shadow appears to be magnified in supine position.[apspjcaserep.com]

Treatment

  • Management and treatment Early diagnosis and surgical treatment are mandatory in order to prevent cardiac failure and pulmonary hypertension.[orpha.net]
  • With early treatment such as surgery, most babies do well and can expect a normal life. Routine follow-ups with a heart specialist (cardiologist) will be needed.[mountsinai.org]
  • If the baby does not have treatment they most likely not survive their first year. However, those who do survive without treatment are likely to have severe complications, or die suddenly during the following years.[chd-uk.co.uk]

Prognosis

  • Prognosis Patients treated early in life have good short and long-term outcome. If left untreated, the disease may lead to congestive cardiac failure and pulmonary hypertension, with 30% of infants dying within 3 months.[orpha.net]
  • Treatment and prognosis Prognosis depends significantly on the extent of collateral formation, however, most infants die within the 1 st year of birth 4.[radiopaedia.org]
  • Awareness of this condition is essential for prompt diagnosis and referral to a cardiac center for early surgical intervention and improved prognosis. This article reviews this rare but serious disease in children.[jfcmonline.com]
  • Treatment and prognosis Repositioning of the artery usually reverses compression, particularly when the underlying tracheobronchial tree is normal.[radiopaedia.org]

Etiology

  • Etiology The pathogenesis still remains unknown, although an association has been suggested with the 22q11.2 deletion syndrome (see this term).[orpha.net]
  • Etiology Either of two etiologies can be responsible for ALCAPA: the bulbus cordis undergoes abnormal septation into the aorta and pulmonary trunk persistence of the pulmonary buds with concomitant involution of the aortic buds that are precursors of[radiopaedia.org]
  • Etiology Theories have been described to explain the origin of a coronary artery from the pulmonary trunk specifically related to the embryologic division of the truncus arteriosus.[omicsonline.org]

Epidemiology

  • Summary Epidemiology The incidence is estimated at 0.1% of all congenital cardiac anomalies and around 200 cases have been described in the world literature.[orpha.net]
  • Chugh SS, Reinier K, Teodorescu C, Evanado A, Kehr E, et al. (2008) Epidemiology of sudden cardiac death: clinical and research implications. ProgCardiovasc Dis 51: 213-228.[omicsonline.org]
  • We present the results of a literature review of the epidemiological, physiopathological, clinical, diagnostic, and surgical characteristics of this rare condition.[revespcardiol.org]
Sex distribution
Age distribution

Pathophysiology

  • Provides comprehensive coverage of physiology, pathophysiology, pharmacology, and nursing management related to care of the critically ill child. Includes detailed Nursing Care Plans for select disorders.[books.google.com]
  • Wesselhoeft H, Fawcett JS, Johnson AL (1968) Anomalous origin of the left coronary artery from the pulmonary trunk: its clinical spectrum, pathology, and pathophysiology, based on a review of 140 cases with seven further cases.[link.springer.com]
  • […] etiologies can be responsible for ALCAPA: the bulbus cordis undergoes abnormal septation into the aorta and pulmonary trunk persistence of the pulmonary buds with concomitant involution of the aortic buds that are precursors of the coronary arteries Pathophysiology[radiopaedia.org]

Prevention

  • Management and treatment Early diagnosis and surgical treatment are mandatory in order to prevent cardiac failure and pulmonary hypertension.[orpha.net]
  • Early repair is carried out to avoid pulmonary vascular disease of the lung.[4] The repair should be contemplated within the first six months of life to prevent severe pulmonary vasculature obstructive disease.[apspjcaserep.com]
  • […] months after surgery. 7 Patients with severe restenosis may require surgical angioplasty, balloon dilatation or stent placement. 7 Anomalous origin of a PA branch from the aorta is a rare entity in which prompt diagnosis and surgery are essential to prevent[revespcardiol.org]
  • The early or late clinical expression reflects the number of collaterals that the patient developed to prevent ischemia and necrosis in the left ventricle.[elsevier.es]
  • Second, financial constraints prevent referral of patients with AORPA to a better hospital.[cardiothoracicsurgery.biomedcentral.com]

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