Cardiology
Section 6: Cardiology
6.2 HEART DISEASES SUBSECTIONS .1 X-rays
6.2.4 Angiography/Pathological Specimen
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Balloon Mitral Valvotomy (BMV)
Figure 6.2.4.3: Balloon mitral valvotomy Photo Courtesy: S Harikrishnan
Angio pictures of progressive dilatation of stenosed mitral valve orifice. The classical dumbbell appearance of the balloon across mitral valve is also seen.
BMV is the procedure of choice in MS in all ages. It has supplanted CMV. The valve area doubles with a minimal risk of MR and the good result lasts for at least 10 years.
Figure 6.2.4.1: Catheterization lab Photo Courtesy: M Zulfikar Ahamed
The photograph shows a diagnostic catheterization lab.
• Diagnostic catheterization in CHD has given way to catheter interventions. Almost all PDAs, more than half of ASDs, a small percent of VSDs and a few AP windows are closed by device. Balloon valvotomy is the treatment of choice in PS, AS, MS and TS. A significant proportion of CoA is managed with Balloon and stent.
• Other uses of catheterization are stenting of PDA, closure of CAVF, MAPCA, Balloon septostomy, etc.
Catheterization Lab
Balloon Dilatation of Pulmonary Valve (BPV)
Figure 6.2.4.2: Balloon dilatation of pulmonary valve
Photo Courtesy: S Sivasankaran
Angio picture shows the process of dilatation of stenotic pulmonary valve by a balloon. The waist is seen, which will be completely abolished once successful dilatation is over.
BPV is quite safe and offers an excellent result in >95% and the result is long lasting. The mortality is near zero. BPV is offered when PS gradient is more than 50 mm Hg.
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Coil Occlusion of PDA
Figure 6.2.4.4: Coil occlusion of PDA Photo Courtesy: S Sivasankaran
Coils are seen (Gianturco or Cook), which are deployed via catheter to close a smaller PDA. Coil closure of PDA is much less expensive than device closure. Occasionally larger PDA is also closed by multiple coils delivered through a bioptome.
Coils are also used in closing collaterals, CAVF, PAVF and other unwanted channels.
Pulmonary AV Fistula (PAVF)
Figure 6.2.4.5: Pulmonary AV fistula Photo Courtesy: S Harikrishnan
Angiogram shows LPA injection opacifying a fistula located at left lower lobe of lung and draining back to LA through pulmonary vein.
The characteristic angio picture is quite diagnostic. PAVF causes central cyanosis with no murmur, normal ECG and near normal X-ray.
Echo anatomy of the heart also will be reported as normal. Rarely it may cause a continuous murmur and may cast a definite shadow in the lung.
Treatment is either by resection of fistula, resection of the particular lung lobe, tying off the feeder vessel or coil embolization of feeding vessel.
Septal Occluder (Amplatzer Device)
Figure 6.2.4.6: Septal occluder (Amplatzer Device)
Photo Courtesy: S Sivasankaran
Both ASD and VSD can be closed nonsurgically by the septal occluder device. It is made of Nitinol, which is a metal with a memory so that when introduced through a catheter over a defect, it will assume its original shape and close the defect appropriately.
Devices can be used to close ASD, VSD (muscular), PDA, AP window, etc. There is recently a device introduced for closing perimembranous VSD also.
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Figure 6.3.1: Atrial futter with 4:2 and 6:2 AV Block
Photo Courtesy: M Zulfikar Ahamed
The saw-toothed appearance with varying AV block. It can cause irregularly irregular pulse. This was found in a newborn. Atrial flutter in newborn or infants can be associated with Ebstein anomaly. It can be idiopathic also, as was in this case.
Treatment is by IV b-Blocker, IV amiodarone or DC version.
Adenosine does not work in atrial flutter.
Complete Heart Block
Figure 6.3.2: Complete heart block Photo Courtesy: M Zulfikar Ahamed
There is AV dissociation (varying PR intervals—there is no regular relationship between P and QRS), atrial rate of 75/mt and ventricular rate of 45/mt and narrow QRS complex. Most likely it is suprahisian block.
CHB can be a medical emergency.
If so IV isoprenaline infusion can be tried. One may require temporary pacing. PPI is offered based on specific indications.
Atrial Flutter with 4:2 and 6:3 AV Block
6.3 EMERGENCIES
Pathology Specimen Showing Single Ventricle
Figure 6.2.4.7: Pathology specimen showing single ventricle
Photo Courtesy: S Sankar, PN Manju, C Indrani
There is only one ventricle without any intervening septum. Single ventricle is an admixture lesion at ventricular level. This can exist with PAH or PS. Single ventricle with PS will behave like TOF.
Single ventricle is an uncommon but very important CCHD. Single ventricle with PAH will present with CHF and mild cyanosis and is managed by PA banding followed by Fontan operation. SV with PS will present with cyanosis and is managed with Fontan with or without prior Glenn shunt.
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Obstructed TAPVC
Figure 6.3.4: Obstructed TAPVC Photo Courtesy: M Zulfikar Ahamed
This is the classical appearance of
‘white washed’ lung and is a very important differential diagnosis of HMD. By CXR it is very difficult to differentiate between the two. White washed lung is due to severe PVH.
Obstructed TAPVC is a genuine cardiac emergency. Obstructed TAPVC with cyanosis becomes bad on PGE 1. Emergency surgery should be done for all obstructed TAPVC.
SVT
Figure 6.3.5: SVT
Photo Courtesy: M Zulfikar Ahamed
Regular tachycardia with a rate of 300/mt in a newborn. QRS is narrow and there are retrograde P waves in II, III AVF. Most likely SVT is due to AVRT—due to an accessory pathway. In newborn, SVT can present with CHF, shock and extreme irritability.
Termination is by IV adenosine.
Nonresponsive SVT can be terminated by IV amiodarone. In an unstable baby, cardio version is employed (0.5–1.0 J/kg).
d-TGA in the Newborn
Figure 6.3.3: d-TGA in the newborn Photo Courtesy: Balu Vaidyanathan
In the echo, note that the posterior left ventricle gives rise to a branching, broad vessel—the pulmonary artery. RV is large and gives rise to aorta. d-TGA is the most common CCHD in the newborn and survival at 1 year without surgery is only 10%. d-TGA usually presents in the first week of newborn period with deep cyanosis and mild CHF.
Immediate management is by oxygen, prostaglandin E1 (PGE1) and balloon atrial septostomy (BAS). BAS is also called Rashkind procedure and is a life-saving procedure. Arterial switch operation (ASO) is offered as early as possible, at least before 4 weeks. Late
presentation of TGA will require Senning operation.
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DiGeorge Syndrome
Figures 6.4.2A and B: DiGeorge syndrome Photo Courtesy: VH Sankar, M Zulfikar Ahamed
Fluorescence in situ hybridization (FISH) showing 22q deletion and echo showing Truncus arteriosus.
DiGeorge syndrome (Catch 22) is a syndrome inherited in autosomal recessive manner. There is a 22q deletion. It is associated with conotruncal anomalies—Truncus arteriosus, interrupted aortic arch, TOF and DORV. In fact , up to 10 to 15% of TOF will have DiGeorge syndrome by FISH.
The syndrome will have associated hypocalcemia and immunodeficiency and hence all conotruncal anomalies of the heart require testing for DiGeorge syndrome. Preoperative work-up is needed for CHD undergoing surgery. Counseling is also offered to mothers, who were operated for TOF, when they become pregnant.