Order Venous Malformations Discussion Questions

Order Venous Malformations Discussion Questions
Order Venous Malformations Discussion Questions
1. An overweight teenager with a history of tetralogy of Fallot
(TOF) returns to the clinic for the rst time after several
years. He underwent complete repair as an infant. He
complains of fatigue and tires easily with modest effort.
He is cyanotic, and nail beds are clubbed. Pulse oximetry
reveals an O2
sat of 80%. A transthoracic echo is performed
but is limited secondary to body habitus. The echo does
demonstrate right ventricular enlargement (RVE), no evidence of intracardiac shunting, mild tricuspid regurgitation
with a peak gradient of 23 mm Hg, and mild pulmonary
A transesophageal echo with saline contrast injection into a
left-hand vein is performed. See Videos 37.1 to 37.3.
What is the most likely diagnosis?
A. Severe pulmonary hypertension
B. Residual inferior atrial septal defect and left SVC
communicating to the left atrium
C. Severe pulmonary insufciency and subsequent right
heart enlargement
D. Pulmonary arteriovenous malformations
E. Partial anomalous pulmonary venous return
2. An echocardiogram is performed on a 29-year-old female
secondary to a murmur (see Videos 37.4 to 37.6). Which of the
following is least commonly associated with this nding(s)?
A. A peripherally inserted central catheter placed in the
left basilic vein will take an atypical course.
B. Progressive right heart failure.
C. Left ventricle enlargement.
D. Pulmonary hypertension.
E. A large left-to-right shunt.
3. A 37-year-old female presents with dyspnea on exertion.
Pulse oximetry reveals normal O2
saturation. A chest x-ray
reveals mesocardia, a hazy right lung eld with probable
right lung hypoplasia, and a vertical density noted from
the right heart border to just below the right diaphragm.
Order Venous Malformations Discussion Questions
An echocardiogram with limited imaging is performed
(see Videos 37.7 and 37.8).
The most likely diagnosis is:
A. Pulmonary hypertension
B. Eventration of right diaphragm
C. Pulmonary arteriovenous malformations of the
right lung
D. Scimitar syndrome
E. Tracheoesophageal stula with chronic aspiration
4. Videos 37.9 to 37.11 are obtained on an outpatient 18-yearold referred secondary to a heart murmur. Based on your
interpretation of the following images, this patient is most
at risk for which of the following?
A. Intracranial berry aneurysms
B. Fulminant sepsis secondary to Streptococcus
C. Cirrhosis
D. Early-onset atherosclerosis
E. Pulmonary embolism
Answer 1: B.
Option A: False. Tricuspid regurgitation gradient was normal
at 23 mm Hg.
Option B: Correct. See Discussion.
Option C: False. There was only mild PI so this is not likely
to be etiology of RVE.
Option D: False. The echo reveals an ASD and RVE. In the
case of pulmonary AVMs, a saline contrast venous injection
will ll the right heart rst with quick return to the LA. In this
case, the LA lls rst and then shunts left to right over the ASD.
Option E: False. PAPVR does cause RVE but is an overall
left-to-right shunt, and therefore patients with PAPVR are not
Tetralogy of Fallot (TOF) is the most common congenital cyanotic heart defect. Complete repair typically occurs in
infancy and consists of ventricular septal defect closure, right
ventricular outšow tract muscle bundle resection, and relief
of pulmonary valve stenosis—most commonly via a transannular patch. Associated lesions, such as an atrial septal defect
(ASD), are typically repaired—although occasionally, a patent
foramen ovale is left open to promote left ventricular lling
(via right-to-left shunting) and maintain cardiac output in neonatal repairs or in cases of severe right ventricular hypertrophy.
The most feared sequela in adult patients following TOF repair
is the risk of ventricular arrhythmias and sudden death, which
has been correlated to RV overload secondary pulmonary
insufciency. Following complete and successful TOF repair,
Chapter 37
Venous Malformations
Brent J. Barber
(c) 2015 Wolters Kluwer. All Rights Reserved.
316 Section VIII Congenital Heart Disease and Fetal Echocardiography
patients will have normal oxygen saturation and therefore will
not be clubbed or cyanotic. The TEE images reveal a large
inferior ASD with left-to-right shunt, which accounts for the
RV enlargement in this patient. Inferior ASDs, located near
the IVC, can be difcult to diagnose via TTE secondary to
their posterior location and in this case were missed by previous echocardiograms and even by the surgeon during repair!
Defects at the level of the IVC—sinus venosus type—are rare
and are associated with partial anomalous venous return.
This case presents with another rare venous anomaly not
typically associated with TOF—that of a left SVC (LSVC)
communicating directly to the left atrium. A persistent LSVC
is an embryologic remnant of the cardinal venous system. A
persistent LSVC typically drains to the coronary sinus—but in
this case drained directly to the left atrium.
Saline contrast echocardiography is a safe and effective tool
to aid in the diagnosis of intra- and extracardiac shunts including systemic venous abnormalities
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