Age: 17 years
Gender: Male
Occupation: High school student
Working diagnosis: Double-inlet left ventricle
HISTORY
Soon after birth, the patient developed heart failure and was diagnosed with double-inlet LV with ventriculoarterial discordance (transposition). Heart failure was due to volume overload from increased pulmonary flow. He was treated medically until he underwent banding of the pulmonary trunk at the age of 1 year. When he was 10 years old, he was referred for further surgical treatment. His mean pulmonary arterial pressure was 37 mm Hg with a Qp/Qs value of 1.58, so Fontan repair was not an option. Eventually, ventricular septation was chosen as a definitive repair and was performed uneventfully.
While he did reasonably well for some time on diuretics with supplemental albumin infusions and afterload reduction therapy, his physical abilities gradually became more limited. He developed dependent edema. At age 17, he developed severe edema. Echocardiography showed progressive and severe regurgitation across the right-sided AV valve.
In addition to having edema and ascites, the patient had mild diarrhea. Hypoalbuminemia was detected, and an intestinal scintigram revealed the evidence of gastrointestinal protein loss. He was diagnosed as having PLE. Several treatment options were employed with little alleviation of his symptoms.
He had repeated hospitalizations for treatment of congestive failure, and had quite severe exercise intolerance. He was reinvestigated in anticipation of further discussion about potential interventions.
Comments: In hearts with double-inlet LV, both the left-sided and the right-sided AV valves insert to the morphologically left and dominant ventricle. The morphologic RV is usually small and incomplete, lacking its inlet portion.
There are two types of double-inlet LV. The more common type, with the aorta arising anteriorly from the hypoplastic RV and the pulmonary trunk arising from the dominant LV (ventriculoarterial discordance), was present in this patient. This is often designated as [S, L, L] type according to Van Praagh’s classification. Without pulmonary stenosis (or banding), pulmonary vascular resistance will become too high to allow for Fontan correction. The alternative arrangement, in which the aorta arises from the dominant LV and the pulmonary trunk arises from the hypoplastic RV (ventriculoarterial concordance), is known as Holmes heart.
Ventricular septation, an uncommon procedure, involves the creation of a ventricular septum using a patch. Heart block is common because the course of the conduction bundles is very close to the intended suture line for attaching the patch. To minimize this probability, the surgeon may omit placing a deep stitch at the likely crossing point of the conduction axis, though this may result in a small residual baffle leak and left-to-right shunt. Such was the case in this patient, as seen later at catheterization.
The dominant LV was dilated (240% of a normal LV). The inlet valves had separate orifices with no tendinous cords shared. If the ventricular volume was smaller than 200% of normal, or if the inlet valves had some of the tension apparatus shared or had a common orifice, ventricular septation would not have been elected.
PLE is known to occur when right heart function is severely impaired and when systemic venous pressure remains chronically high. The lymphatic drainage system of the intestine is congested. This problem is encountered most frequently in Fontan patients (see Case 62 ). For quantitative evaluation of PLE, alpha-1-antitrypsin measurement in the stool is the test of choice.
In double-inlet LV, the AV valves are often structurally abnormal. In some hearts, both of the inlet valves have two leaflets, and, in others, they are bilaterally trifoliate. Either valve can be regurgitant.
CURRENT SYMPTOMS
The patient was severely restricted by dyspnea in performing his usual activities. He became breathless walking up one flight of steps and often had to stop half way. Frequent hospitalizations were interrupting his school work. He did not have palpitations, dizziness, lightheadedness, or chest pain.
NYHA class: III
CURRENT MEDICATIONS
Enalapril 2.5 mg daily
Furosemide 80 mg daily
Spironolactone 150 mg daily
Potassium L-aspartate 1800 mg daily
Digoxin 0.2 mg daily
Chlorthiazide 2 mg daily
Denopamine 20 mg daily
Coenzyme Q10, 30 mg daily
Comments: Denopamine is a partial beta-1 agonist developed and tried in Japan. Its sympathomimetic action has been used for the treatment of coronary vasospasm and heart failure.
PHYSICAL EXAMINATION
BP 100/70 mm Hg, HR 85 bpm, regular rhythm, oxygen saturation 97%
Height 155 cm, weight 45 kg, BSA 1.39 m 2
Neck veins: Jugular veins could not be assessed.
Lungs/chest: Sounds were clear with no rales.
Heart: There was a 3/6 pan-systolic murmur at the fourth left intercostal space.
Abdomen: Abdomen was soft and flat, mild hepatomegaly.
Extremities: Lower extremities were edematous to the shins.
Comments: We expected the JVP to be elevated, but could not confirm this. Absence of a large V-wave may not necessarily exclude right AV valve regurgitation if the atrium is large and extremely compliant. The murmur was likely due to right or perhaps left AV valve regurgitation, or could represent residual left-to-right shunt through a leak in the VSD patch.
LABORATORY DATA
| WBC | 5.34 µ10 3 /µL (4.0–9.0) |
| Hemoglobin | 14.9 g/dL (13.0–17.0) |
| Hematocrit/PCV | 44.0% (41–51) |
| Platelet count | 246 x 10 9 /L (150–350) |
| Sodium | 135 mmol/L (138–145) |
| Potassium | 3.6 mmol/L (3.4–4.9) |
| Creatinine | 0.4 mg/dL (0.7–1.3) |
| Blood urea nitrogen | 11 mg/dL (6–24) |
| Total protein | 4.4 g/dL (6.5–8.2) |
| Albumin | 2.7 g/dL (3.6–5.5) |
| Globulin | 1.7 g/dL (2.0–3.0) |
| A/G | 1.6 (1.3–2.5) |
| Total bilirubin | 0.5 mg/dL (0.2–1.2) |
| Direct bilirubin | 0.2 mg/dL (0–0.4) |
| Indirect bilirubin | 0.3 mg/dL (0.2–0.8) |
| AST | 21 U/L (0–40) |
| ALT | 23 U/L (0–35) |
| ALP | 83 U/L (32–97) |
| GTP | 101 U/L (13–68) |
| LDH | 160 U/L (100–225) |
| Triglycerides | 208 mg/dL (30–130) |
| HDL-cholesterol | 24 mg/dL (40–55) |
| Uric acid | 6.1 mg/dL (3.8–8.0) |
| Ca | 8.1 mg/dL (8.8–10.5) |
| BNP | 75.6 pg/mL (<20.0) |
Comments: Many of the abnormalities present are consistent with PLE, including hypoproteinemia and hypoalbuminemia. The elevated BNP level indicates cardiac failure.
ELECTROCARDIOGRAM
FINDINGS
Heart rate: 112 bpm
QRS axis: +74°
QRS duration: 108 msec
Atrial tachycardia. Diffuse ST changes.
Holter ECG: Sinus rhythm with occasional atrial and ventricular ectopics.
Comments: The patient had a past history of transient atrial tachycardia recorded when he was 15 years old. Because such nonsustained arrhythmias can be recurrent and clinically silent, ambulatory ECG monitoring is often prudent. Persistent tachycardia can depress ventricular function.
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