#congenital-heart see also: [Cameron's Congenital heart disease](x-devonthink-item://E383FFBD-9FD8-4DE9-B350-0F6F60D3E600?page=515) [Congenital heart disease](x-devonthink-item://50AB3061-1565-4C26-AB58-7D63A0FA1A01) [rosen congenital heart disease](x-devonthink-item://11EB751F-77A9-4ED9-94E1-7A3863F059F1?page=6) [Congenital Heart disease PBL focus question](x-devonthink-item://ED564D25-952A-4F1E-BE88-1CE168BD1C95) [armando Hasudugan - congenital heart defect](https://armandoh.org/disease/congenital-heart-defect/) [Single ventricle defects and the hunt for the best shunt - Don't Forget the Bubbles](cubox://card?id=7200433261116394509) ```dataview Table FROM #congenital-heart SORT file.name ASC ``` > [!key points] > - cyanotic lesions generally present early > - neonates with duct-dependent lesions are often well in early neonatal period and collapse at around 4-7 days with closure of the ductus arteriosus > - commonest duct-dependent left-sided obstructive lesions are coarctation of the aorta, critical aortic stenosis, hypoplastic left-heart syndrome, and interrupted aortic arch > **Lack of response to 100% oxygen for at least 10 minutes** (hyperoxygenation) indicates that the etiologic origin for the clinical picture is likely cardiac, and further cardiac workup is indicated. > - Hyperoxygenation will help infants with pulmonary parenchymal etiologic origins and those with pulmonary hypertension as causes of cyanosis. A cyanotic newborn who is otherwise well appearing may also have a hemoglobinopathy, such as methemoglobinemia. > - Chest radiography and echocardiography performed to rule out cyanotic CHD are indicated in a cyanotic infant who is not responsive to hyperoxygenation. > - *hepatomegaly* suggest cardiac involvement **most common lesions presenting in the first week of life** - critical aortic stenosis - coarctation of the aorta - large VSD - AVSD - truncus arteriosus others less common at this age: - transposition of great vessels - hypoplastic left heart syndrome - ToF - tricuspid atresia - pulm atresia # 3 common clinical presentations - *cyanotic* → ↓ pulm blood flow due to RV obstruction lesions/hypoplasia , mixing of deoxygenated (pulmonary circulation) and oxygenated (systemic circulation) blood - *congestive heart failure* → Left to right shunting and consequent pulm congestion - *shock* → obstructive lesion after lesion eg aortic coarctation resulting in ↓ stroke volume and shock # cyanotic vs acyanotic | Cyanotic (T's) | Acynotic (D's) | | --------------------------------------------------- | ----------------------------------------------------------- | | **T**etralogy of Fallot | AS**D** | | **T**ransposition of the great vessles | VS**D** | | **T**ricuspid atresia | AVS**D** | | **T**runcus arteriosus | Patent **D**uctus Arteriosus | | **T**otal anomalous pulmonary venous return (TAPVR) | **Other:** Pulmonary stenosis, Aortic stenosis, Coarctation | # Ductal dependent lesions > Present two ways (generally present in neonate; other CHD present a bit late with signs of congestive heart failure) > **Pulmonary blood flow** → cyanosis, > **Systemic blood flow** → shock > > indication for [[prostaglandin]] There are [2 types of ductal-dependent defects](x-devonthink-item://AA741F67-03BE-4E4D-B585-B251654C3C9F?page=4&start=859&length=35&search=2%20types%20of%20ductal-dependent%20defects). 1. Obstructive lesions of the **right side of the heart** (e.g., *tricuspid atresia*, *pulmonary atresia*, *severe pulmonary stenosis*) ==require an open DA to provide adequate **pulmonary** blood flow.== As the DA begins to close, obstructive right heart lesions will develop *cyanosis* 2. Obstructive lesions of the **left side of the heart** (e.g., *hypoplastic left ventricle*, *coarctation of the aorta*, *aortic stenosis*) ==require the DA to maintain adequate **systemic** blood flow==. Obstructive left heart lesions will develop hypoperfusion, *shock*, and pulmonary edema. 1. generally not very responsive to IV fluids cyanotic: TGA, TOF/critical pulm stenosis, tricuspid/pulm atresia shock: coarctation, aortic stenosis ## pulmonary valve stenosis - insufficient pulmonary blood flow to lungs - needs PDA to get blood to lungs ## transposition of great vessels with intact atrial septal defect - cont murmour - "egg on string" CXR ## coarctation of the aorta - insufficient systemic blood flow ## hypoplastic left heart syndrome - insufficient systemic blood flow # Congestive heart failure in infants **respiratory symptoms** - wheezing - retractions - tachyponea - difficulty feeding - *hepatomegaly* # flow chart ![[Pasted image 20230824234914.png]] # Related Questions ## congenital heart disease - [ ] 1Q: [16 day old, unwell & unresponsive](x-devonthink-item://D4C19F6F-0718-4AD7-BDC4-34B460451B98?page=16) -- [Answer](x-devonthink-item://736EC9CD-AC9C-4588-BA1E-F4AD190CBA47?page=22) - [ ] 2Q: [Cardiac Kid](x-devonthink-item://EB2A381E-D8D3-4236-B51F-2DF81CE08885?page=6) -- [Answer](x-devonthink-item://8455B512-D9BD-4314-927B-D3208619EE2A?page=6)