Age: 34 years
Gender: Female
Occupation: Homemaker
Working diagnosis: Partial anomalous pulmonary venous connection
HISTORY
The patient was well at birth and throughout childhood, but had been told her heart was on the right side and that she had one pulmonary vein draining to the RA, but that this would not cause her problems. As a teenager, she had sporadic episodes of scant hemoptysis.
One year ago the patient became pregnant for the first time, and she had two episodes of cough with small amounts of bright red blood. She had no other symptoms and had no further recurrence of hemoptysis throughout the duration of her successful pregnancy.
More recently she complained of increased airway secretions and cough after exposure to second-hand smoke and described seeing bright red blood mixed with her sputum. Several days later she suddenly had another episode of hemoptysis, not precipitated by prolonged coughing, and a second similar episode later the same day. In each case, she described expectorating bright red blood with a small blood clot.
The patient was a nonsmoker and had been generally healthy in other ways.
Comments: Patients with isolated partial anomalous pulmonary venous connection (PAPVC) are usually asymptomatic early in life, and the anomaly can often go unrecognized. When new symptoms occur, any known congenital heart abnormality ought to be considered for its potential role in the patient’s problem, as demonstrated throughout this book.
Isolated hemoptysis is an uncommon presentation of PAPVC. Because of the presence of a congenital vascular abnormality, hemoptysis may in theory also be due to rupture of a small vessel (or of a collateral) into the airway. More likely, however, abnormalities of the pulmonary veins may coexist with congenital abnormalities of the bronchial tree or bronchial arteries. These can result in small airways with limited vascular supply that are prone to recurrent infection.
Because the most common cause of hemoptysis in any patient is chronic bronchitis this may be a likely scenario explaining this woman’s current problem. The amount of bleeding in this patient is not life threatening, and the recurrent nature of her low-volume hemoptysis suggests bleeding from a bronchial arteriole rather than the larger pulmonary arteries.
Hemoptysis can be associated with pulmonary arterial hypertension, although this patient’s history does not suggest this.
CURRENT SYMPTOMS
Apart from hemoptysis, the patient had no other symptoms. She had not had an upper respiratory illness. She denied any chest discomfort or shortness of breath, and had a good exercise tolerance. She was able to climb three flights of stairs without difficulty. There had been no increase in abdominal girth or lower extremity edema. She denied palpitations or syncope.
Comments: Lack of symptoms is the rule in PAPVC. Patients who develop symptoms (dyspnea on exertion, atrial arrhythmia, or, later in life, right heart failure and pulmonary hypertension) usually have more than one anomalously connected pulmonary vein and/or associated cardiac lesions. Symptoms often relate to the magnitude of left-to-right shunt and/or the onset of atrial tachyarrhythmia.
NYHA class: I
PHYSICAL EXAMINATION
BP 100/68 mm Hg, HR 62 bpm, respirations 10, oxygen saturation 98% on room air
Height 142 cm, weight 64 kg, BSA 1.59 m 2
Oropharynx: The oropharynx was moist, nonerythematous, and without blood, postnasal drip, or exudates.
Nasal mucosa: The mucosa was slightly hyperemic without telangiectasia, blood, or polyps.
Neck veins: Her JVP was < 3 cm above the sternal angle, with a normal waveform.
Lungs/chest: Auscultation of the chest was normal.
Heart: The point of maximal impulse was in the right precordium in the fourth intercostal space just medial to the right midclavicular line. There was no RV heave. Heart sounds were regular with a normal first and second heart sound. No murmurs or gallops were heard.
Abdomen: There was normal abdominal situs. The abdomen was soft and nontender and without hepatomegaly.
Extremities: Extremities were warm and without edema, clubbing, or cyanosis.
PERTINENT NEGATIVES
There were no petechiae, ecchymoses, or telangiectasias.
Comments: The resting oxygen saturation is normal and there is no clubbing or cyanosis to suggest chronic hypoxia.
It is important to exclude oropharyngeal and nasal sources of bleeding in the setting of hemoptysis.
The cardiac impulse is rightward. A RV heave with wide splitting of S2 may be appreciated with significant PAPVC and right heart dilation. A prominent pulmonary second heart sound may also be heard if the pulmonary artery pressures are elevated.
Approximately 25% of patients presenting with PAPVC have an associated cardiac malformation, most often an ostium secundum or sinus venosus atrial septal defect. Other associated lesions include ventricular septal defect, patent ductus arteriosus, coarctation of the aorta, tetralogy of Fallot, double outlet RV, mitral stenosis or atresia, pulmonary stenosis, and aortic stenosis.
Hemoptysis and telangiectasia may be seen in hereditary hemorrhagic telangiectasia.
LABORATORY DATA
| Hemoglobin | 14.6 g/dL (11.5–15.0) |
| Hematocrit | 43% (36–46) |
| MCV | 87 fL (83–99) |
| Platelet count | 198 × 10 3 µ/L (150–400) |
| Creatinine | 0.8 mg/dL (0.6–1.2) |
| Blood urea nitrogen | 4.9 mmol/dL (2.5–6.5) |
Comments: The hemoglobin and hematocrit are normal, as would be expected for only scant hemoptysis.
ELECTROCARDIOGRAM
FINDINGS
Heart rate: 62 bpm
QRS axis: −30°
QRS duration: 110 msec
Sinus rhythm, normal intervals, and P-wave axis. Prominent anterior forces. Leftward QRS axis.
Comments: The ECG is almost entirely normal except for the high R-wave voltages in the early precordial leads. These reflect the relative position of the heart in the right chest.
The classic findings of dextrocardia, namely inverted P-wave in lead I, right axis deviation, and reversed precordial R-wave progression, are not seen. The patient more likely may have only dextroposition of the heart.
CHEST X-RAY
FINDINGS
Dextroposition, atrial situs solitus, and a left-sided aortic arch were seen. A large anomalous connecting vein (scimitar vein) was present in the right mid and lower lung zones. The trachea was deviated to the right. There was volume loss of the right lung and hyperinflation of the left lung.
Comments: The “scimitar sign,” first described in 1949, derives from the shadow of the anomalous pulmonary venous connection (scimitar vein) on posteroanterior CXR, which resembles a Turkish sword known as a scimitar.
Right lung hypoplasia, a characteristic component of scimitar syndrome, causes rightward traction on the trachea and compensatory left lung hyperinflation. The heart also shifts rightward in the mediastinum.
The bronchial anatomy is normal (the left bronchus has a longer course before branching), which indicates normal atrial situs, and usually normal abdominal situs.
EXERCISE TESTING
| Exercise protocol: | Modified Bruce |
|---|---|
| Duration (min : sec): | 13 : 45 |
| Reason for stopping: | Fatigue |
| ECG changes: | None |
| Rest | Peak | |
|---|---|---|
| Heart rate (bpm): | 62 | 164 |
| Percent of age-predicted max HR: | 88 | |
| O 2 saturation (%): | 98 | 100 |
| BP (mm Hg): | 100/68 | 184/86 |
| Double product: | 30,176 | |
| Peak V o 2 (mL/kg/min): | 38.4 | |
| Percent predicted (%): | 76 | |
| Ve/V co 2 : | 28 | |
| Metabolic equivalents: | 15.2 | |
Stay updated, free articles. Join our Telegram channel
Full access? Get Clinical Tree
