Presentation
Hypoplastic right-heart syndrome presenting as multiple miscarriages. Texas Heart Institute Journal, 39 (2), 249-254. [mayoclinic.pure.elsevier.com]
[…] if the condition was present at the time of inpatient admission. [icd.codes]
Herein, we describe the case of a 23-year-old woman who presented for evaluation of repeated miscarriages and was found to have hypoxemia and erythrocytosis. Further evaluation revealed hypoplastic right-heart syndrome with an intracardiac shunt. [ncbi.nlm.nih.gov]
Present On Admission POA Help "Present On Admission" is defined as present at the time the order for inpatient admission occurs — conditions that develop during an outpatient encounter, including emergency department, observation, or outpatient surgery [icd10data.com]
Presenting the clinical picture of the hypoplastic right heart syndrome. ( 4224286 ) Morgan A.D....Shanklin D.R. 1966 [malacards.org]
Entire Body System
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Hypoxemia
The erythrocytosis and hypoxemia resolved, and she was able to conceive and deliver a healthy baby at term 2 years later. [ncbi.nlm.nih.gov]
These infants are usually very unstable, with severe hypoxemia (not enough oxygen in the blood) and acidosis (too much acid in the blood), complicating treatment. [cincinnatichildrens.org]
After birth, due to decreased pulmonary vascular resistance and closure of the ductus arteriosus, infants with HLHS develop a constellation of symptoms that include cyanosis, dyspnea, heart failure, hypoxemia and acidosis. [cardiovascularultrasound.biomedcentral.com]
This may result in hypoxemia. A delicate balance between pulmonary and systemic vascular resistances should be maintained to ensure adequate oxygenation and tissue perfusion. [emedicine.medscape.com]
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Cyanotic Congenital Heart Disease
Hypoplastic right heart syndrome is a rare cyanotic congenital heart disease with under-development of the right ventricle, tricuspid, and pulmonary valves leading to right-to-left shunting of the blood through inter-atrial septal defect. [ncbi.nlm.nih.gov]
Psychiatrical
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Fear
These children and their parents are in constant fear that today may be the day they lose their child. They fear that today maybe the day that they go into heart failure. [change.org]
We went through the rollercoaster of emotions along the way, from fear, anger, frustration, guarded joy when she was born by induction at St Michael’s in Bristol, to abject despair and grief, through determination, fear again and finally being totally [patchesheartgroup.org]
I was 20 when my daughter was born, and went through all the emotions and fears of surgery and health and the future. She doesn't have the same problem as your little bubby, but i do understand, the feelings and emotions you go through. [circleofmoms.com]
Workup
Serum
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Erythrocytosis
The erythrocytosis and hypoxemia resolved, and she was able to conceive and deliver a healthy baby at term 2 years later. [ncbi.nlm.nih.gov]
Treatment
Timely and appropriate treatment led to a successful pregnancy after repeated miscarriages. [mayoclinic.pure.elsevier.com]
Many children with congenital heart defects don't need treatment, but others do. Treatment can include medicines, catheter procedures, surgery, and heart transplants. [icdlist.com]
This rare anomaly requires prenatal diagnosis since it needs immediate and emergency treatment. [en.wikipedia.org]
Prognosis
Prostoglandin infusion to keep the ductus open has improved the prognosis. [fetalultrasound.com]
Long-term prognosis for children who undergo the Fontan procedure is good. They are expected to live 15-30 years before needing an additional surgery or transplant. Adult heart transplants are far more readily available than newborn transplants. [theclarkfirmtexas.com]
Treatment and prognosis HLHS can be well-tolerated in-utero due to the fetal right ventricle being the dominant chamber and the ductus arteriosus being patent. [radiopaedia.org]
The prognosis after this operation for single ventricle patients is better than for other treatments, and improvements of surgical technique continue to be made. [pted.org]
Etiology
Certain conditions have both an underlying etiology and multiple body system manifestations due to the underlying etiology. [icd10coded.com]
Abortion, Spontaneous/etiology Congenital/complications/physiopathology/therapy/surgery Heart defects Treatment outcome Ventricular function, Right/physiology Cardiology and Cardiovascular Medicine Cite this Apa Standard Harvard Vancouver BIBTEX RIS Dib [mayoclinic.pure.elsevier.com]
Pulmonary atresia with or without ventricular septal defect: a different etiology and pathogenesis for the atresia in the 2 types? Am J Cardiol 1983;51:932:935. Hornberger LK, Sanders SP, Sahn DJ, Rice, MJ, Spevak PJ, Benacerraf BR. [iame.com]
Multifactorial inheritance hypothesis for etiology of congenital heart disease: the genetic-environmental interaction. Circulation. 1968. 38:604-17. Lev M, Arcilla R, Rimoldi HJ, Licata RH, Gasul BM. [emedicine.medscape.com]
Epidemiology
[…] cause is misalignment of the atrial septum to the left. [18] More recent studies suggest that hypoplastic left heart syndrome is genetically heterogeneous and hypoplastic left heart syndrome and bicuspid aortic valve are genetically related. [19, 20] Epidemiology [emedicine.medscape.com]
Pathophysiology
The difference in pulmonary diameter is likely due to the pathophysiology of the lesion. In the presence of a VSD, the small pulmonary trunk is due to a congenital cardiac anomaly. [iame.com]
Severely hypoplastic left ventricle can be present in hearts with double-outlet right ventricle and common atrioventricular canal; in some studies, these variants constitute as many as 25% of hypoplastic left heart syndrome cases. [12] Pathophysiology [emedicine.medscape.com]
Prevention
While we cannot control or prevent CHD or GI complications, we can all try to improve our education about nutrition and dental preventative care, both of which can have a direct impact on children with CHD. [hypoplasticrighthearts.org]
The medications were to strengthen his heart, prevent fluid retention, help with reflux, and prevent blood clots since he now had a Gortex shunt placed in his heart. [cardiackids.wordpress.com]
Prevention There are no current guidelines to prevent HRHS because the cause is unknown. If you are pregnant, proper prenatal care may help decrease the risk of having a baby with a heart defect. [winchesterhospital.org]
What about preventing endocarditis? Children with HLHS are at increased risk for developing endocarditis. Ask your pediatric cardiologist about your child's need to take antibiotics before certain dental procedures to help prevent endocarditis. [heart.org]
There's no known cause of hypoplastic left heart syndrome, so there's no known way to prevent the condition. [mayoclinic.org]