Age: 54 years old
Gender: Female
Occupation: Housewife
Working diagnosis: Partial anomalous pulmonary venous return
HISTORY
The patient was well throughout her life until the age of 44 (10 years previously) when she developed paroxysmal atrial fibrillation and congestive heart failure. Her symptoms were controlled with diuretics. Workup included an echocardiogram showing normal LV function but biatrial enlargement and a dilated RV. There were no valvular abnormalities and no pulmonary hypertension suggested by the echocardiogram.
Further workup was pursued with cardiac catheterization, which demonstrated that the right upper pulmonary vein (RUPV) was abnormally connected to the superior caval vein. The atrial septum was intact. There was mild pulmonary hypertension (mean of 31 mm Hg), but the pulmonary vascular resistance was not elevated. The calculated Qp/Qs was 1.2. At that time, surgery was not recommended. She had been doing well on diuretics since then.
Her heart failure worsened 9 years after the initial episode. Her symptoms required hospitalization on several occasions, so that further workup was pursued.
Comments: In this patient, an abnormal RUPV was found. Because the Qp/Qs ratio was believed to be close to normal (if the calculations were true, why is the RV dilated?), and because the patient’s symptoms resolved easily with treatment, no intervention was felt to be necessary (see Case 6 ).
PHYSICAL EXAMINATION
BP 130/70 mm Hg, HR 58 bpm and irregular, oxygen saturation 97%
Height 152 cm, weight 65 kg, BSA 1.66 m 2
Neck veins: Severely engorged (12 cm above the sternal angle), with large V-waves present
Lungs/chest: Clear
Heart: The rhythm was irregularly irregular. There was a left parasternal lift. There was a 1/6 systolic murmur at the third left intercostal space, whereas the second heart sound had an increased pulmonary component, but was normally split.
Abdomen: Normal
Extremities: There was bilateral 2+ pitting ankle edema.
Comments: The neck veins reflect tricuspid regurgitation. The clinical examination suggests that the RV dilation reported 9 years ago was still present and clinically relevant.
The second heart sound was normally split. Although this does not necessarily exclude an ASD, the absence of a fixed and wide splitting of the second heart sound is noteworthy and other causes must be explored. In this case, the only potential cause was the isolated RUPV anomaly diagnosed previously.
LABORATORY DATA
| Hemoglobin | 12.3 g/dL (12.0–16.5) |
| Hematocrit/PCV | 38.0% (35.0–45.0) |
| Platelet count | 405 × 10 9 /L (150–350) |
| Sodium | 142 mmol/L (138–145) |
| Potassium | 3.8 mmol/L (3.4–4.9) |
| Creatinine | 0.5 mmol/dL (0.5–1.0) |
| Blood urea nitrogen | 12 mg/dL (8–20) |
| Total protein | 6.7 g/dL (6.5–8.2) |
| Albumin | 3.8 g/dL (3.6–5.5) |
Comments: No specific abnormalities present.
ELECTROCARDIOGRAM
FINDINGS
Heart rate: 59 bpm
QRS axis: +52°
QRS duration: 90 msec
Atypical atrial fibrillation, nonspecific ST changes
A 24-hour ECG was also performed. The heart rate ranged from 40 to 108 bpm. There were rare PVCs, and no long pauses.
Comments: Atrial fibrillation reflects long-standing right atrial enlargement. What the ECG does not demonstrate here is RV hypertrophy. The QRS axis does not suggest that the RV is dilated, even though the history and physical exam point to RV overload being present.
The 24-hour ECG can be helpful in determining whether appropriate rate control is present.
CHEST X-RAY
FINDINGS
Cardiothoracic ratio: 70%
Large cardiac silhouette with prominent central pulmonary arteries, and increased vascularity on the right side. The left heart border was straight, compatible with right ventricular dilation. The left main bronchus was horizontal, suggesting LA enlargement.
Comments: Significant cardiomegaly here argues for long-standing volume overload from the shunt. The finding is incompatible with the previously reported Qp/Qs ratio of only 1.2 : 1.
ECHOCARDIOGRAM
FINDINGS
The LV was normal in size with normal systolic function. The LA was mildly enlarged. The RA and RV were both enlarged, the atrial septum was intact, and no abnormal pulmonary venous drainage could be demonstrated in this study.
Comments: RA and RV enlargement are signs of significant volume overload. This is not surprising here given the CXR.
What the echo does not show is an ASD. The atrial septum was well visualized and intact. In this type of setting, unexplained RV enlargement without visible shunt, the examiner should consider a TEE or a bubble study to exclude a sinus venosus ASD, and a TEE, MRI, or CT angiogram to evaluate possible anomalous pulmonary venous connections (see Case 1 ).
FINDINGS
There was moderate to severe tricuspid regurgitation (peak velocity mildly increased at 3.0 m/sec). The tricuspid valve appeared structurally normal.
Comments: The clinician may be tempted to suggest that the tricuspid regurgitation is the cause of the RV and RA enlargement, yet the valve itself was structurally normal. Because RV enlargement was described on an echocardiogram 9 years ago, it argues that the tricuspid valve regurgitation is secondary to chamber enlargement.
The patient importantly does not have pulmonary hypertension.
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